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laboratory studies: Hemoglobin 4gtdL(4OglL) Which of the following is the most appropriate additional Leukoc5rte count 6oO0/pL (6 x lOe/L) treatment during chemotherapy? Mean corpuscular volume 90 fL (A) Epoetin Platelet count 450,000/pL (450 x 10e/L) (B) Fluconazole Reticulocyte count <0.1% of erythrocytes (C) Metronidazole Serum lactate dehydrogenase, haptoglobin, and total (D) Valacyclovir bilirubin levels are normal. Parvovirus polymerase chain reaction and antibody testing are positive. Bone marrow biopsy reveals a marked reduction in erythroid precursors and giant proerythro- Item 5 blasts with intranuclear viral inclusions. A 42-year-old woman is evaluated for several months of fatigue. She reports a normal diet and no gastrointestinal Which of the following is the most appropriate treatment? symptoms. Medical history is notable for hypothyroidism and vitiligo. Her only medication is levothyroxine. (A) Acyclovir On physical examination, vital signs are normal. She (B) Erythropoietin has several well-deflned, hypopigmented macules sym- (C) Intravenous immune globulin metrically distributed on the extremities and trunk. (D) Prednisone Iaboratory studies: Hemoglobin 8.8 g/dl (88 g/L) Leukocyte count 4000/pL (4 x 10e/L) Item 3 Mean corpuscular volume tt7 fL A 27-year-old woman is evaluated for shortness of breath Platelet count 160,000/pL (160 x 10e/L) and palpitations. She reports no chest pain, cough, or Reticuloc5rte count 1% oferythrocytes 71

Self-Assessment Test t/t (D ? D Peripheral blood smear is shown. (C) Erythrocyteexchange transfusion UI (D) Intravenousfurosemide UI rD vt UI .D Item 7 A 4S-year old man is evaluated as part of routine annual .D examination. Medical history is signiflcant for hyperlipid- UI emia. He has a 28-pack-year history of cigarette smoking. His only medication is pravastatin. On physical examination, vital signs and other flnd ings are normal. taboratory studies: Hemoglobin t4.6 gtdL (146 gtL) Leukocyte count 12,500/pL (12.5 x l0e/L) (80% neutrophils, 15% lymphocytes, 5'1, monocl.tes) Absolute neutrophil count 10,000/pL (10 x l0'/L) Platelet count 302,000/pL (gOZ x rOelL) The peripheral blood smear is unremarkable

.D Item 7 A 4S-year old man is evaluated as part of routine annual .D examination. Medical history is signiflcant for hyperlipid- UI emia. He has a 28-pack-year history of cigarette smoking. His only medication is pravastatin. On physical examination, vital signs and other flnd ings are normal. taboratory studies: Hemoglobin t4.6 gtdL (146 gtL) Leukocyte count 12,500/pL (12.5 x l0e/L) (80% neutrophils, 15% lymphocytes, 5'1, monocl.tes) Absolute neutrophil count 10,000/pL (10 x l0'/L) Platelet count 302,000/pL (gOZ x rOelL) The peripheral blood smear is unremarkable Which of the following is the most likely cause of the patient's elevated leukocyte count? Which ofthe following is the most appropriate initial (A) Chronic myeloid leukemia diagnostic test? (B) Occult infection (A) Homocysteine level (C) Pravastatin (B) Methylmalonic acid level (D) Smoking (C) Serum folate level (D) Serum vitamin B,, level Item 8 A 29-year-oldwoman is evaluated in the emergency depart tr tr Item 6 A 27-year-old woman is hospitalized fbr progressive short- ment for epistaxis and bleeding gums that began 3 days ago. Medical history is otherwise unremarkable. and she takes ness of breath, fever, and chest pain ol 2 days' duration. no medications. Medical history is significant for sickle cell anemia (hemo- On physical examination, temperature is 36.7 'C globin SS disease) with frequent pain crises. Medications (98.0 'F). blood pressure is 110180 mm Hg, pulse rate is are hydroxyurea, folic acid, and methadone. 120/min, and respiration rate is 22trnirr. Dried blood is seen On physicalexamination. temperature is 38.4'C (101.1 'F), in her nose, and gingival bleeding is noted. No lymphade- blood pressure is 104/67 mm Hg, pulse rate is 122lmin. and nopathy or hepatosplenomegaly is present. Petechiae are respiration rate is 24lmin. Oxygen saturation is 92'2, breathing present on the lower extremities. 40'1, oxygen lry face mask. No jugular venous distention is present. Cardiopulmonary examination reveals a grade 216 Laboratory studies: systolic ejection murrnur without extra cardiac sounds and Hemoglobin 8.e g/dl (8e gi L) bilateral crackles in the lower lung. No edema is present. Leukocy.te count 14,0001pL (14 x t}e I L) (19"/,, neutrophils, 3'2, bands. 32'1, Laboratory studies: lymphocltes. 15'X, monocytes. and Hemoglobin 7.t gtdL (71 gll.) 31'X, "atypical" cells) Leukocyte count 15.400/pL (15.4 x 10'q/L) Platelet count 8000/pL (8 x 10erL) Platelet count 225,000/pL (22.5 x 10"/L) Reticulocyte count 12'2, of erythrocytes The prothrombin time, activated partial thromboplas tin time, and INR are elevated; flbrinogen level is low. and Bilateral areas of new segmental consolidation are fibrin degradation products are elevated. seen on the chest radiograph. Peripheral blood smear is shown (top of next page). Incentive spirometry, ceftriaxone and azithromycin. and prophylactic low molecular-weight heparin are initiated. Which of the following is the most likely diagnosis? Which ofthe following is the most appropriate additional (A) Acute lymphoblastic leukemia management? (B) Acute promyelocytic leukemia (A) Cardiac cilt heterization (C) Aplastic anemia (B) CT angiography (D) Primary myelofibrosis

Which of the following is the most likely cause of the patient's elevated leukocyte count? Which ofthe following is the most appropriate initial (A) Chronic myeloid leukemia diagnostic test? (B) Occult infection (A) Homocysteine level (C) Pravastatin (B) Methylmalonic acid level (D) Smoking (C) Serum folate level (D) Serum vitamin B,, level Item 8 A 29-year-oldwoman is evaluated in the emergency depart tr tr Item 6 A 27-year-old woman is hospitalized fbr progressive short- ment for epistaxis and bleeding gums that began 3 days ago. Medical history is otherwise unremarkable. and she takes ness of breath, fever, and chest pain ol 2 days' duration. no medications. Medical history is significant for sickle cell anemia (hemo- On physical examination, temperature is 36.7 'C globin SS disease) with frequent pain crises. Medications (98.0 'F). blood pressure is 110180 mm Hg, pulse rate is are hydroxyurea, folic acid, and methadone. 120/min, and respiration rate is 22trnirr. Dried blood is seen On physicalexamination. temperature is 38.4'C (101.1 'F), in her nose, and gingival bleeding is noted. No lymphade- blood pressure is 104/67 mm Hg, pulse rate is 122lmin. and nopathy or hepatosplenomegaly is present. Petechiae are respiration rate is 24lmin. Oxygen saturation is 92'2, breathing present on the lower extremities. 40'1, oxygen lry face mask. No jugular venous distention is present. Cardiopulmonary examination reveals a grade 216 Laboratory studies: systolic ejection murrnur without extra cardiac sounds and Hemoglobin 8.e g/dl (8e gi L) bilateral crackles in the lower lung. No edema is present. Leukocy.te count 14,0001pL (14 x t}e I L) (19"/,, neutrophils, 3'2, bands. 32'1, Laboratory studies: lymphocltes. 15'X, monocytes. and Hemoglobin 7.t gtdL (71 gll.) 31'X, "atypical" cells) Leukocyte count 15.400/pL (15.4 x 10'q/L) Platelet count 8000/pL (8 x 10erL) Platelet count 225,000/pL (22.5 x 10"/L) Reticulocyte count 12'2, of erythrocytes The prothrombin time, activated partial thromboplas tin time, and INR are elevated; flbrinogen level is low. and Bilateral areas of new segmental consolidation are fibrin degradation products are elevated. seen on the chest radiograph. Peripheral blood smear is shown (top of next page). Incentive spirometry, ceftriaxone and azithromycin. and prophylactic low molecular-weight heparin are initiated. Which of the following is the most likely diagnosis? Which ofthe following is the most appropriate additional (A) Acute lymphoblastic leukemia management? (B) Acute promyelocytic leukemia (A) Cardiac cilt heterization (C) Aplastic anemia (B) CT angiography (D) Primary myelofibrosis 72

Self-Assessment Test rrt G' Mean corpuscular 110 fL h volume (l, Platelet count 105,000/pL (105 x 10,/L) E Reticulocy.te count 1.57, oferythrocytes tt ta Vitamin B,, Normal (l, sf ut Folate Normal ut Which of the following is the most appropriate test to o vt ( perform next? (A) Bone marrow biopsy (B) Colonoscopy CI (C) Direct antiglobulin test *lr (D) Iron and ferritin levels and total iron-binding capacity ITEM 8

Which of the following is the most appropriate test to o vt ( perform next? (A) Bone marrow biopsy (B) Colonoscopy CI (C) Direct antiglobulin test *lr (D) Iron and ferritin levels and total iron-binding capacity ITEM 8 Item 11 tr Item 9 A 40 year old woman is hospitalized with acute short A 46 year old woman is evaluated fbllowing discovery of' an elevated hemoglobin level. She is asymptomatic, her ness of breath. Medical history is signiflcant tbr tw<-r preg nancy losses in the second trimester. Laboratory testing history is unremarkable, and she takes no medications. 3 months ago after her second pregnancy loss was posi On physical examination, vital signs are normal. The spleen tip is palpable. tive Ibr lupus anticoagulant, high titer IgC anticardiolipin antibodies, and high titer IgG anti p, glycoprotein anti laboratory studies: bodies. She is otherwise well and takes no medications. Erythropoietin 2 mU/mL (2UlL) On physical examination, pulse rate is 120/min Hematocrit 51% and respiration rate is 20/min; oxygen saturation is 92'X, Hemoglobin t7 gtdL(uo gtL) breathing ambient air. Leukocyte count TOoolltL(7 xl}elL) Laboratory studies show a positive lultus anticoagu Platelet count 750,000/pL (750 x 10,/L) lant, high titer IgC anticardiolipin antibodies, and high Genetic testing is positive fbr the lAK2Y6l7F mfia titer lgc anti 92 glycoprotein antibodies. tion. CT angiography shows a pulmonary embolism in the Low-dose aspirin is started. Phlebotomy is scheduled. right main pulmonary artery. Low molecular weight heparin is initiirted. Which of the following is the most appropriate additional management? Which of the following is the most appropriate long-term treatment? (A) Hydroxyurea (A) Dabigatran plus aspirin (B) Imatinib (B) l{ivaroxaban (C) Interferon o (C) Warfarin (D) Ruxolitinib (D) Warfarin plus inf'erior vena cava fllter (E) No further management

Item 11 tr Item 9 A 40 year old woman is hospitalized with acute short A 46 year old woman is evaluated fbllowing discovery of' an elevated hemoglobin level. She is asymptomatic, her ness of breath. Medical history is signiflcant tbr tw<-r preg nancy losses in the second trimester. Laboratory testing history is unremarkable, and she takes no medications. 3 months ago after her second pregnancy loss was posi On physical examination, vital signs are normal. The spleen tip is palpable. tive Ibr lupus anticoagulant, high titer IgC anticardiolipin antibodies, and high titer IgG anti p, glycoprotein anti laboratory studies: bodies. She is otherwise well and takes no medications. Erythropoietin 2 mU/mL (2UlL) On physical examination, pulse rate is 120/min Hematocrit 51% and respiration rate is 20/min; oxygen saturation is 92'X, Hemoglobin t7 gtdL(uo gtL) breathing ambient air. Leukocyte count TOoolltL(7 xl}elL) Laboratory studies show a positive lultus anticoagu Platelet count 750,000/pL (750 x 10,/L) lant, high titer IgC anticardiolipin antibodies, and high Genetic testing is positive fbr the lAK2Y6l7F mfia titer lgc anti 92 glycoprotein antibodies. tion. CT angiography shows a pulmonary embolism in the Low-dose aspirin is started. Phlebotomy is scheduled. right main pulmonary artery. Low molecular weight heparin is initiirted. Which of the following is the most appropriate additional management? Which of the following is the most appropriate long-term treatment? (A) Hydroxyurea (A) Dabigatran plus aspirin (B) Imatinib (B) l{ivaroxaban (C) Interferon o (C) Warfarin (D) Ruxolitinib (D) Warfarin plus inf'erior vena cava fllter (E) No further management Item 10 Item 12 A 67-year-old woman is evaluated for fatigue and dys- A 32 year old woman is evaluated for dyspnea with exer- pnea on exertion occurring for the past 2 months. Medical tion of 2 weeks' duration. Medical history is signiflcant history is significant for breast cancer 15 years ago, which for systemic lupus erythematosus. Family history is unre was treated with surgery, chemotherapy, and radiation. markable. Her only medication is hydroxychloroquine. Her history is otherwise unremarkable, and she takes no On physical examination, blood pressure is 98/60 mm medications. Hg and pulse rate is 107/min; other vital signs are normal. On physical examination, vital signs are normal. Oxy Oxygen saturation is 98% breathing ambient air. Other gen saturation is 97"/u breathing ambient air. Skin pallor is than tachycardia, the physical examination is normal. noted. Abdominal examination reveals no hepatospleno Laboratory studies: megaly, petechiae, or bruising. Haptoglobin Undetectable taboratory studies: Hemoglobin e.B g/dL (eB g/L) Hemoglobin 7.7 gtdL(T etr) Leukocyte count 42OOl1tL(4.2xt}e lL) Leukocyte count 3600/pL (3.6 x 10'q/L) (35% Platelet count 125,000/pL (12s x 1oe/L) neutrophils, 23% lymphocytes, Reticulocyte count 15'1, of erythrocytes

Item 10 Item 12 A 67-year-old woman is evaluated for fatigue and dys- A 32 year old woman is evaluated for dyspnea with exer- pnea on exertion occurring for the past 2 months. Medical tion of 2 weeks' duration. Medical history is signiflcant history is significant for breast cancer 15 years ago, which for systemic lupus erythematosus. Family history is unre was treated with surgery, chemotherapy, and radiation. markable. Her only medication is hydroxychloroquine. Her history is otherwise unremarkable, and she takes no On physical examination, blood pressure is 98/60 mm medications. Hg and pulse rate is 107/min; other vital signs are normal. On physical examination, vital signs are normal. Oxy Oxygen saturation is 98% breathing ambient air. Other gen saturation is 97"/u breathing ambient air. Skin pallor is than tachycardia, the physical examination is normal. noted. Abdominal examination reveals no hepatospleno Laboratory studies: megaly, petechiae, or bruising. Haptoglobin Undetectable taboratory studies: Hemoglobin e.B g/dL (eB g/L) Hemoglobin 7.7 gtdL(T etr) Leukocyte count 42OOl1tL(4.2xt}e lL) Leukocyte count 3600/pL (3.6 x 10'q/L) (35% Platelet count 125,000/pL (12s x 1oe/L) neutrophils, 23% lymphocytes, Reticulocyte count 15'1, of erythrocytes 21% monocytes, no immature Lactate dehydrogenase 475 U IL leukocyte forms) Peripheral blood smear shows spherocytes. 73

Self-Assessment Test ln .D Which of the following is the most appropriate diagnostic On physical examinltion, vital signs and examination lrt test to perform next? findings are normal. ta |D (A) ADAMTS13 activity and inhibitor u) laboratory studies: ur (B) Bone marrow biopsy Activated partial 37 s .D (C) Direct antiglobulin test thromboplastin time (aPTT) aPTT rt ith mixing study 26 s (D) Osmotic fragility test (D Hematocrit 30')1, Platelet count UI 190.000,9L (190 x 10" L) Prothronrbin time 11 s Item 13 Platelet function testirlg is normal. A 4S-year-old man is hospitalized for neutropenic fever. He was recently diagnosed with diffuse large B-cell lym- phoma, and his last chemotherapy treatment was 10 days Which of the following is the most appropriate diagnostic ago. His course of chemotherapy consisted of rituximab, test to perform nefi? cyclophosphamide, doxorubicin, vincristine (day 1), and (A) Dilute Russell viper venom time prednisone (days l-5). He is treated appropriately with a (B) Measure tactor levels broad-spectrum antibiotic and is discharged 5 days later feeling improved and with a recovering neutrophil count. (C) Platelet aggregation study (D) von Willebrand antigen assay Which of the following is the most appropriate management to reduce the risk of future febrile neutropenia? (A) Reduce cyclophosphamide and doxorubicin doses Item 16 A 4s-year old man is evaluated for an acute stroke. He tr was hospitalized 48 hours ago lollolving a motor vehicle (B) Start granulocyte colony-stimulating factor (G-CSF) accident in which he sustained several fractures requiring weekly internal fixation. Further surgery is scheduled. Tu'ent)' (C) Start G-CSF at the time of chemotherapy four hours ago. he u'as diagnosed rl ith a pulmonary embo (D) Start levofloxacin at the time of chemotherapy lism. and futl dose unfiactionated heparin lvas started. Neurologic symptoms have developed over the past 30 minutes. Before hospitalization, he was taking no Item 14 medications. A 28-year-old woman is seen to establish care. Medical his On physical eramination. temperature is normal. tory is signiflcant for acute lymphoblastic leukemia diag- blood pressure is 110160 mm Hg. pulse rate is 100'min, nosed and treated at age 5 years; she has been leukemia and respiration rate is 16i min. Oxygen saturation is 94'X, free since completion of therapy that included anthracy breathing oxygen, 2 L/min. The patient is awake with nor cline and high-dose glucocorticoids. Bone mineral density mal langu:rge function. No papilledema is present. Pupils measurement following treatment was normal. An echo- are equal and reactive. Left facial weakness. dysarthria, cardiogram performed l year ago was normal. She reports and flaccid paralysis in the left arm are noted. regular menses. She has no medical problems and takes no Noncontrast CT scan shows an acute intracerebral medications. She is not sexually active and does not smoke hemorrhage, I cm in diameter. in the right basal ganglia cigarettes, use recreational drugs, or drink alcohol. She without intraventricular hemorrhage or midline shift. exercises 150 minutes per week. Unfiactionated heparin is discontinued. The physical examination is unremarkable. BMI is 28. Cervical cancer screening is performed. Which of the following is the most appropriate additional Complete blood count is normal. treatment? (A) Cryoprecipitate Which of the following is the most appropriate additional (B) ldarucizumab survivorship assessment to perform next? (C) Protamine sulfate (A) Bone marrow biopsy (D) Vitamin K (B) Estrogen and progesterone levels (C) Exercise stress test (D) Lipids and fasting glucose Item 17 (E) Whole genome sequencing A 32-year-old woman is seen for follow-up evaluation of chronic anemia that has been stable for at least the past 5 years. She has no history of transfusions. She had one tr Item 15 A 25 year-old woman is hospitalized fbr bleeding 1 day pregnancy at age 29 years, ending in intrauterine fetal demise in the early third trimester. Her father has anemia. following a tonsillectomy. She reports a history of heavy The patient is Chinese. menses since menarche but no spontaneous bruising or On physical examination, vital signs and the remain muscle or joint hematomas. der of the physical examination are normal.

ln .D Which of the following is the most appropriate diagnostic On physical examinltion, vital signs and examination lrt test to perform next? findings are normal. ta |D (A) ADAMTS13 activity and inhibitor u) laboratory studies: ur (B) Bone marrow biopsy Activated partial 37 s .D (C) Direct antiglobulin test thromboplastin time (aPTT) aPTT rt ith mixing study 26 s (D) Osmotic fragility test (D Hematocrit 30')1, Platelet count UI 190.000,9L (190 x 10" L) Prothronrbin time 11 s Item 13 Platelet function testirlg is normal. A 4S-year-old man is hospitalized for neutropenic fever. He was recently diagnosed with diffuse large B-cell lym- phoma, and his last chemotherapy treatment was 10 days Which of the following is the most appropriate diagnostic ago. His course of chemotherapy consisted of rituximab, test to perform nefi? cyclophosphamide, doxorubicin, vincristine (day 1), and (A) Dilute Russell viper venom time prednisone (days l-5). He is treated appropriately with a (B) Measure tactor levels broad-spectrum antibiotic and is discharged 5 days later feeling improved and with a recovering neutrophil count. (C) Platelet aggregation study (D) von Willebrand antigen assay Which of the following is the most appropriate management to reduce the risk of future febrile neutropenia? (A) Reduce cyclophosphamide and doxorubicin doses Item 16 A 4s-year old man is evaluated for an acute stroke. He tr was hospitalized 48 hours ago lollolving a motor vehicle (B) Start granulocyte colony-stimulating factor (G-CSF) accident in which he sustained several fractures requiring weekly internal fixation. Further surgery is scheduled. Tu'ent)' (C) Start G-CSF at the time of chemotherapy four hours ago. he u'as diagnosed rl ith a pulmonary embo (D) Start levofloxacin at the time of chemotherapy lism. and futl dose unfiactionated heparin lvas started. Neurologic symptoms have developed over the past 30 minutes. Before hospitalization, he was taking no Item 14 medications. A 28-year-old woman is seen to establish care. Medical his On physical eramination. temperature is normal. tory is signiflcant for acute lymphoblastic leukemia diag- blood pressure is 110160 mm Hg. pulse rate is 100'min, nosed and treated at age 5 years; she has been leukemia and respiration rate is 16i min. Oxygen saturation is 94'X, free since completion of therapy that included anthracy breathing oxygen, 2 L/min. The patient is awake with nor cline and high-dose glucocorticoids. Bone mineral density mal langu:rge function. No papilledema is present. Pupils measurement following treatment was normal. An echo- are equal and reactive. Left facial weakness. dysarthria, cardiogram performed l year ago was normal. She reports and flaccid paralysis in the left arm are noted. regular menses. She has no medical problems and takes no Noncontrast CT scan shows an acute intracerebral medications. She is not sexually active and does not smoke hemorrhage, I cm in diameter. in the right basal ganglia cigarettes, use recreational drugs, or drink alcohol. She without intraventricular hemorrhage or midline shift. exercises 150 minutes per week. Unfiactionated heparin is discontinued. The physical examination is unremarkable. BMI is 28. Cervical cancer screening is performed. Which of the following is the most appropriate additional Complete blood count is normal. treatment? (A) Cryoprecipitate Which of the following is the most appropriate additional (B) ldarucizumab survivorship assessment to perform next? (C) Protamine sulfate (A) Bone marrow biopsy (D) Vitamin K (B) Estrogen and progesterone levels (C) Exercise stress test (D) Lipids and fasting glucose Item 17 (E) Whole genome sequencing A 32-year-old woman is seen for follow-up evaluation of chronic anemia that has been stable for at least the past 5 years. She has no history of transfusions. She had one tr Item 15 A 25 year-old woman is hospitalized fbr bleeding 1 day pregnancy at age 29 years, ending in intrauterine fetal demise in the early third trimester. Her father has anemia. following a tonsillectomy. She reports a history of heavy The patient is Chinese. menses since menarche but no spontaneous bruising or On physical examination, vital signs and the remain muscle or joint hematomas. der of the physical examination are normal. 74

Self-Assessment Test vt taboratory studies: at 16 weeks' gestation. Medications include only prenatal F6' Hemoglobin 10.8 g/dl (108 e/L) vitamins. E Mean corpuscular volume 62fL On physical examination, vital signs are normal. Dried o Reticulocyte count 2% of erythrocytes blood is noted around the nares. She has a gravid uterus. E UI Ut Iron studies laboratory studies: o U} Ferritin 200 ng/ml (200 pg/L) Hemoglobin 72.2gldL(l22glL) U! lron 2oo ltgldL (eo pmol/L) Leukocyte count 4500/pL (4.5 x 10e/L) + Total iron binding capacity 280 pg/dl (SO pmol/L) a, Platelet count 66,000/pL (66 x 10'g/L) vt Hemoglobin electrophoresis reveals a normal migra- Creatinine 0.7 mg/dl (0f.s pmol/L) tion pattern of hemoglobin A and normal hemoglobin A, and hemoglobin F levels. Peripheral blood smear shows normal erythrocytes Peripheral blood smear is shown. and granulocytes without platelet clumping.

vt taboratory studies: at 16 weeks' gestation. Medications include only prenatal F6' Hemoglobin 10.8 g/dl (108 e/L) vitamins. E Mean corpuscular volume 62fL On physical examination, vital signs are normal. Dried o Reticulocyte count 2% of erythrocytes blood is noted around the nares. She has a gravid uterus. E UI Ut Iron studies laboratory studies: o U} Ferritin 200 ng/ml (200 pg/L) Hemoglobin 72.2gldL(l22glL) U! lron 2oo ltgldL (eo pmol/L) Leukocyte count 4500/pL (4.5 x 10e/L) + Total iron binding capacity 280 pg/dl (SO pmol/L) a, Platelet count 66,000/pL (66 x 10'g/L) vt Hemoglobin electrophoresis reveals a normal migra- Creatinine 0.7 mg/dl (0f.s pmol/L) tion pattern of hemoglobin A and normal hemoglobin A, and hemoglobin F levels. Peripheral blood smear shows normal erythrocytes Peripheral blood smear is shown. and granulocytes without platelet clumping. c i i":.:

vt taboratory studies: at 16 weeks' gestation. Medications include only prenatal F6' Hemoglobin 10.8 g/dl (108 e/L) vitamins. E Mean corpuscular volume 62fL On physical examination, vital signs are normal. Dried o Reticulocyte count 2% of erythrocytes blood is noted around the nares. She has a gravid uterus. E UI Ut Iron studies laboratory studies: o U} Ferritin 200 ng/ml (200 pg/L) Hemoglobin 72.2gldL(l22glL) U! lron 2oo ltgldL (eo pmol/L) Leukocyte count 4500/pL (4.5 x 10e/L) + Total iron binding capacity 280 pg/dl (SO pmol/L) a, Platelet count 66,000/pL (66 x 10'g/L) vt Hemoglobin electrophoresis reveals a normal migra- Creatinine 0.7 mg/dl (0f.s pmol/L) tion pattern of hemoglobin A and normal hemoglobin A, and hemoglobin F levels. Peripheral blood smear shows normal erythrocytes Peripheral blood smear is shown. and granulocytes without platelet clumping. c i i":.: .-Srii - :.. : Which of the following is the most likely diagnosis for her thrombocytopenia? (A) Gestationalthrombocytopenia ' l. l'{..:"i (B) HELLP syndrome (C) Immunethrombocytopenicpurpura {i} (D) Thrombotic thrombocytopenic purpura

.-Srii - :.. : Which of the following is the most likely diagnosis for her thrombocytopenia? (A) Gestationalthrombocytopenia ' l. l'{..:"i (B) HELLP syndrome (C) Immunethrombocytopenicpurpura {i} (D) Thrombotic thrombocytopenic purpura Item 2O A 49-year-old man is evaluated for progressive fatigue and a 13.6-kg (30-lb) weight loss in 4 months. Medical history is unremarkable, and he takes no medications. On physical examination, vital signs are normal. On abdominal palpation, the spleen is enlarged to 17 cm. taboratory studies: Which of the following is the most likely diagnosis? Hemoglobin trgtdL (110 g/L) (A) a Thalassemia carrier Leukocyte count 87,500/pL (87.s x 1oe/L) (877. neutrophils, 2% bands, 2% (B) cr Thalassemia trait metamyelocytes, 3% myeloblasts, (C) 0 Thalassemia minor 2% basophils, 2% lymphocytes) (D) Hemoglobin E trait Platelet count 465,000/pL (465 x 10e/L) Bone marrow biopsy shows hypercellular marrow with granulocytic hyperplasia and 6% myeloblasts. Chro Item 18 mosomal analysis and fluorescence in-situ hybridization A l9-year-old woman is evaluated following a recent diag- studies show t(9;22). nosis of hereditary spherocytosis. She is asymptomatic. She takes no medications. Which of the following is the most appropriate treatment? On physical examination, vital signs are normal. The spleen tip is palpable. (A) Hydroxyurea Laboratory studies show a hemoglobin level of 11.7 g/dl (B) Imatinib (ll7 glL), mean corpuscular hemoglobin concentration of (C) Inductionchemotherapy 40 gldL (400 g/L), and reticulocyte count of 6% of eryth- (D) Stemcelltransplantation rocytes. Peripheral blood smear shows frequent spherocytes and polychromasia of the erythrocl'tes and reticulocytosis.

Item 2O A 49-year-old man is evaluated for progressive fatigue and a 13.6-kg (30-lb) weight loss in 4 months. Medical history is unremarkable, and he takes no medications. On physical examination, vital signs are normal. On abdominal palpation, the spleen is enlarged to 17 cm. taboratory studies: Which of the following is the most likely diagnosis? Hemoglobin trgtdL (110 g/L) (A) a Thalassemia carrier Leukocyte count 87,500/pL (87.s x 1oe/L) (877. neutrophils, 2% bands, 2% (B) cr Thalassemia trait metamyelocytes, 3% myeloblasts, (C) 0 Thalassemia minor 2% basophils, 2% lymphocytes) (D) Hemoglobin E trait Platelet count 465,000/pL (465 x 10e/L) Bone marrow biopsy shows hypercellular marrow with granulocytic hyperplasia and 6% myeloblasts. Chro Item 18 mosomal analysis and fluorescence in-situ hybridization A l9-year-old woman is evaluated following a recent diag- studies show t(9;22). nosis of hereditary spherocytosis. She is asymptomatic. She takes no medications. Which of the following is the most appropriate treatment? On physical examination, vital signs are normal. The spleen tip is palpable. (A) Hydroxyurea Laboratory studies show a hemoglobin level of 11.7 g/dl (B) Imatinib (ll7 glL), mean corpuscular hemoglobin concentration of (C) Inductionchemotherapy 40 gldL (400 g/L), and reticulocyte count of 6% of eryth- (D) Stemcelltransplantation rocytes. Peripheral blood smear shows frequent spherocytes and polychromasia of the erythrocl'tes and reticulocytosis. Which of the following is the most appropriate treatment? Item 21 A 68 year clld man is evaluated for a transfusion reaction. tr lle was hospitalized fbr acute upper gastrointestinal bleed (A) Folate ing. While receiving his first unit of'packed red blood cells, (B) Prednisone he developed f'ever, dyspnea, and {lank pain 15 minutes into the transfusion. 'lhe translusion was stopped by the (C) Rituximab nursing staf I. His only rnedication is omeprazole. (D) Splenectomy On physicat examination, temperature is 38.3 'C (100.9 'F), blood pressure is 80/40 mm Hg, pulse rate is 120/min. and respiration rate is 18/min. Oxygen saturation Item 19 is 96'X, breathing ambient air. Cardiopulmonary exam A 28-year-old woman undergoes evaluation for easy ination reveals clear lungs, normaI heart sounds, and low bruising, epistaxis, and bleeding gums. She is pregnant central venous pressure.

Which of the following is the most appropriate treatment? Item 21 A 68 year clld man is evaluated for a transfusion reaction. tr lle was hospitalized fbr acute upper gastrointestinal bleed (A) Folate ing. While receiving his first unit of'packed red blood cells, (B) Prednisone he developed f'ever, dyspnea, and {lank pain 15 minutes into the transfusion. 'lhe translusion was stopped by the (C) Rituximab nursing staf I. His only rnedication is omeprazole. (D) Splenectomy On physicat examination, temperature is 38.3 'C (100.9 'F), blood pressure is 80/40 mm Hg, pulse rate is 120/min. and respiration rate is 18/min. Oxygen saturation Item 19 is 96'X, breathing ambient air. Cardiopulmonary exam A 28-year-old woman undergoes evaluation for easy ination reveals clear lungs, normaI heart sounds, and low bruising, epistaxis, and bleeding gums. She is pregnant central venous pressure. 75

Self-Assessment Test tt .D ln t^ .D tr CONI. Inspection of a collected urine specimen reveals a pink coloration. 5 years ago. She has no other medical problems and takes no medications. On physical examination, vital signs are normal. UI U) Which of the following is the most likely diagnosis? No temporal or jaw tenderness is noted. Proximal girdle muscles are tender to palpation, with restricted range of (D (A) Acute hemolytic transfusion reaction motion owing to stiffness and pain. Muscle strength is (B) Allergic reaction symmetric and normal. No joint effusions or synovitis is .D U) (C) Iransfusion associated circulatory overload evident. (D) Transfusion related acute lung injury laboratory studies: Erythrocyte sedimentation 90 mmih rate llem 22 Hemoglobin 8.s gidl (8s g/L) A 48 year-old woman is evaluated for easy bruising. She Leukocyte count B000ipL (8 x loe/L) with has no history of gingival bleeding, menorrhagia, or bleed- normal differential ing following procedures. Medical history is notable only Platelet count 4s0,o0o/pL (450 x 10e/L) for systemic lupus erythematosus. Medications are predni Mean corpuscular volume 84fL sone, hydroxychloroquine, and NSAIDs as needed. Reticulocyte count l% oferythrocytes On physical examination, vital signs and examination Iron studies flndings are normal. Ferritin 800 ng/ml (800 pg/L) Laboratory studies show an activated partial throm Iron 1o pg/dl (2 pmol/L) boplastin time of 38 seconds, platelet count of 190,000/pL Total iron binding capacity 200 pg/dl (36 pmol/L) (190 x 10e/L), and prothrombin time of 12.5 seconds.

ln t^ .D tr CONI. Inspection of a collected urine specimen reveals a pink coloration. 5 years ago. She has no other medical problems and takes no medications. On physical examination, vital signs are normal. UI U) Which of the following is the most likely diagnosis? No temporal or jaw tenderness is noted. Proximal girdle muscles are tender to palpation, with restricted range of (D (A) Acute hemolytic transfusion reaction motion owing to stiffness and pain. Muscle strength is (B) Allergic reaction symmetric and normal. No joint effusions or synovitis is .D U) (C) Iransfusion associated circulatory overload evident. (D) Transfusion related acute lung injury laboratory studies: Erythrocyte sedimentation 90 mmih rate llem 22 Hemoglobin 8.s gidl (8s g/L) A 48 year-old woman is evaluated for easy bruising. She Leukocyte count B000ipL (8 x loe/L) with has no history of gingival bleeding, menorrhagia, or bleed- normal differential ing following procedures. Medical history is notable only Platelet count 4s0,o0o/pL (450 x 10e/L) for systemic lupus erythematosus. Medications are predni Mean corpuscular volume 84fL sone, hydroxychloroquine, and NSAIDs as needed. Reticulocyte count l% oferythrocytes On physical examination, vital signs and examination Iron studies flndings are normal. Ferritin 800 ng/ml (800 pg/L) Laboratory studies show an activated partial throm Iron 1o pg/dl (2 pmol/L) boplastin time of 38 seconds, platelet count of 190,000/pL Total iron binding capacity 200 pg/dl (36 pmol/L) (190 x 10e/L), and prothrombin time of 12.5 seconds. Which of the following is the most appropriate Which of the following is the most appropriate diagnostic management of this patient's anemia? test? (A) Bone marrow biopsy (A) Factor VIII inhibitor titer (B) Colonoscopy (B) Factor Xl level (C) Erythropoiesis stimulatingagent (C) Factor XII level (D) Low dose prednisone (D) Mixing study (E) Oral iron replacement

Which of the following is the most appropriate Which of the following is the most appropriate diagnostic management of this patient's anemia? test? (A) Bone marrow biopsy (A) Factor VIII inhibitor titer (B) Colonoscopy (B) Factor Xl level (C) Erythropoiesis stimulatingagent (C) Factor XII level (D) Low dose prednisone (D) Mixing study (E) Oral iron replacement Item 23 A 51 year-old woman is evaluated before hospital dis Item 25 charge. She was diagnosed with high-risk acute myeloid A l9-year old woman is evaluated for easy bruising of leukemia and completed induction chemotherapy. Her 2 weeks' duration. She has no other symptoms, and med- leukemia is believed to be secondary to breast cancer ther ical history is unremarkable. She takes no medications. apy, which included surgery and chemotherapy following On physical examination, vital signs are normal. diagnosis 2 years ago. Examination flndings are limited to petechiae on the lower On physical examination, vital signs and other find extremities and small, scattered ecchymoses. ings are normal. A peripherally inserted central catheter is Laboratory studies show a platelet count of 15,000/pL located in the left upper extremity. (15 x lOe/L); the remainder of the complete blood count is Complete blood count and bone marrow aspirate and normal. biopsy indicate compleLe remission. The peripheral blood smear is shown. Which of the following is the most appropriate management?

Item 23 A 51 year-old woman is evaluated before hospital dis Item 25 charge. She was diagnosed with high-risk acute myeloid A l9-year old woman is evaluated for easy bruising of leukemia and completed induction chemotherapy. Her 2 weeks' duration. She has no other symptoms, and med- leukemia is believed to be secondary to breast cancer ther ical history is unremarkable. She takes no medications. apy, which included surgery and chemotherapy following On physical examination, vital signs are normal. diagnosis 2 years ago. Examination flndings are limited to petechiae on the lower On physical examination, vital signs and other find extremities and small, scattered ecchymoses. ings are normal. A peripherally inserted central catheter is Laboratory studies show a platelet count of 15,000/pL located in the left upper extremity. (15 x lOe/L); the remainder of the complete blood count is Complete blood count and bone marrow aspirate and normal. biopsy indicate compleLe remission. The peripheral blood smear is shown. Which of the following is the most appropriate management? 8?tffi% (A) Allogeneic hematopoietic stem cell transplantation (B) Consolidationchemotherapy (C) Intrathecal chemotherapy plus whole brain irradia tion (D) Maintenancechemotherapy (E) No additional treatment Item 24 A 75 year old woman is evaluated for fatigue and a 3-month history of pain in her neck, shoulders, and hips @9@sB @s q'C, -^ and low-grade fevers. She reports no headaches, vision changes, or jaw claudication. Her last colonoscopy was HIV and hepatitis C testing is pending. 76

Self-Assessment Test vt 6' Which ofthe following is the most appropriate management? (A) Glucocorticoids Item 28 A 58 year old man is evaluated in the emergency depart tr Q, (B) Plasma exchange ment for fever, headache. and stiff neck. 'Ihe fever began = UI 2 days ago; the headache and stiff neck were present on UI (C) Platelettransfusion awakening today ancl have become severe. Two weeks ago, (l, tl Ut (D) Observation he was diagnosed with trigeminal neuralgia, and carba- mazepine therapy was initiated. (l, On physical examination. temperature is 38.7'C (101.7 "F), tt

vt 6' Which ofthe following is the most appropriate management? (A) Glucocorticoids Item 28 A 58 year old man is evaluated in the emergency depart tr Q, (B) Plasma exchange ment for fever, headache. and stiff neck. 'Ihe fever began = UI 2 days ago; the headache and stiff neck were present on UI (C) Platelettransfusion awakening today ancl have become severe. Two weeks ago, (l, tl Ut (D) Observation he was diagnosed with trigeminal neuralgia, and carba- mazepine therapy was initiated. (l, On physical examination. temperature is 38.7'C (101.7 "F), tt tr Item 26 A 32 year old man is evaluated in the emergency depart ment for a 1-week history of fatigue, low-grade fever, blood pressure is 110/70 mm I{g, pulse rate is 110/min, and respiration rate is l8/min. Nuchal rigidity is present, but the remainder ol the neurologic examination is otherwise bruising, and epistaxis. He has ncl other medical problems, normal. I Ie has no papilledema. Petechiae and ecchymoses and he takes no medications. are noted over the lower extremities. On physical examination, temperature is ll7.B 'C laboratory studies: (100.0 "F). blood pressure is 120i65 nlm Hg. pulse rate is Activated partial 36s l08/min, and respiration rate is 22lmin. Pallor is noted. thromboplastin time Dried blood is present in the nares. Multiple bruiscs are l laptoglobin Normal seen on his extremities. and he has petechiae at his ankles. Hemoglobin 12.5 gldl (12s g/L) Laboratory studies: Leukocyte count 18,1001p1 (18.1 x 10e/L) Activated partial :]B S Platelet count 30,000/pl. (ao x to"/l-1 thromboplastin time Prothrombin time 11.5 s

tr Item 26 A 32 year old man is evaluated in the emergency depart ment for a 1-week history of fatigue, low-grade fever, blood pressure is 110/70 mm I{g, pulse rate is 110/min, and respiration rate is l8/min. Nuchal rigidity is present, but the remainder ol the neurologic examination is otherwise bruising, and epistaxis. He has ncl other medical problems, normal. I Ie has no papilledema. Petechiae and ecchymoses and he takes no medications. are noted over the lower extremities. On physical examination, temperature is ll7.B 'C laboratory studies: (100.0 "F). blood pressure is 120i65 nlm Hg. pulse rate is Activated partial 36s l08/min, and respiration rate is 22lmin. Pallor is noted. thromboplastin time Dried blood is present in the nares. Multiple bruiscs are l laptoglobin Normal seen on his extremities. and he has petechiae at his ankles. Hemoglobin 12.5 gldl (12s g/L) Laboratory studies: Leukocyte count 18,1001p1 (18.1 x 10e/L) Activated partial :]B S Platelet count 30,000/pl. (ao x to"/l-1 thromboplastin time Prothrombin time 11.5 s D dimer 2.5 pgiml. (2.5 mgr[.) Lactate dehydrogenase Normal I Iematocrit 22"1,, A peripheral blood smear reveals decreased platelets Leukocyte count 2300/pl. (2.3 x 10e/1.) and normal erythrocyte morphology. A direct antiglobulin Platelet count 22,OOO I 1tL (22 x lO" ll .) test is negative. Prothrombin time 25s Carbamazepine is discontinued. Blood cultures are Albumin 3.5 g/dl. (3.s glL) obtained. Dexamethasone and empiric antibiotics are ini Alanine aminotranslerase 30 U/L tiated for presumed bacterial meningitis. Aspartate aminotransferase 35 UIL Bilirubin. total 0.9 mgi dL (15.4 pmol/L) Which of the following is the most appropriate next step in Fibrinogen 60 mg/dl, (0.6 g/t.) management? (A) Lurnbar puncture Which of the following is the most likely diagnosis? (B) Plasma exchange (A) Aplastic anemia (C) Platelet transf usion (B) Coagulopathyofliverdisease (D) No additional interventions (C) Disseminatedintravascularcoagulation (D) Thrornbotic thrombocytopenic purpura Item 29 A 28 year-old wonlan is evaluated for a 3 day history of fever and pharyngitis. Medical history is signiflcant for Item 27 hyperthyroidism diagnosed 3 months ago. She consumes a A 65-year old man is diagnosed with multiple segmental vegetarian diet. Her only medication is methimazole. pulmonary emboli in the right lung. He has no other med On physical examination, temperature is 38.2 'C ical problems, and he takes no medications. (100.8 'F); other vital signs are normal. Posterior pharyn- On physical examination, blood pressure is 132 17 6 mmHg, geal erythema is noted. pulse rate is 100/min, and respiration rate is 18/min. Oxygen Laboratory studies: saturation is 98% at rest breathing ambient air. Hemoglobin 13.9 g/dl (139 g/L) The patient is alert and quickly comprehends the Leukocyte count 23OO I 1tL (2.3 x r}e I L) (2O"L diagnostic implications and required therapy. He does not neutrophils, 7 2% \y mpho - require pain medication. The patient is engaged in a shared cytes, 8'1, monocytes) decision making process regarding treatment. Absolute neutrophil count 460lytL (0.+o x to!'/L) Mean corpuscular volume 92fL Which of the following is the most appropriate Platelet count 302,000/pL (302 x 10e/L) recommendation? (A) Discharge home and treat with dabigatran Which of the following is the most likely diagnosis? (B) Discharge home and treat with rivaroxaban (A) Benign ethnic neutropenia (C) Hospitalize and treat with thrombolytic therapy (B) Cyclic neutropenia (D) Hospitalize and treat with unfractionated heparin and (C) Drug induced neutropenia warfarin (D) Vitamin B,, deliciency

D dimer 2.5 pgiml. (2.5 mgr[.) Lactate dehydrogenase Normal I Iematocrit 22"1,, A peripheral blood smear reveals decreased platelets Leukocyte count 2300/pl. (2.3 x 10e/1.) and normal erythrocyte morphology. A direct antiglobulin Platelet count 22,OOO I 1tL (22 x lO" ll .) test is negative. Prothrombin time 25s Carbamazepine is discontinued. Blood cultures are Albumin 3.5 g/dl. (3.s glL) obtained. Dexamethasone and empiric antibiotics are ini Alanine aminotranslerase 30 U/L tiated for presumed bacterial meningitis. Aspartate aminotransferase 35 UIL Bilirubin. total 0.9 mgi dL (15.4 pmol/L) Which of the following is the most appropriate next step in Fibrinogen 60 mg/dl, (0.6 g/t.) management? (A) Lurnbar puncture Which of the following is the most likely diagnosis? (B) Plasma exchange (A) Aplastic anemia (C) Platelet transf usion (B) Coagulopathyofliverdisease (D) No additional interventions (C) Disseminatedintravascularcoagulation (D) Thrornbotic thrombocytopenic purpura Item 29 A 28 year-old wonlan is evaluated for a 3 day history of fever and pharyngitis. Medical history is signiflcant for Item 27 hyperthyroidism diagnosed 3 months ago. She consumes a A 65-year old man is diagnosed with multiple segmental vegetarian diet. Her only medication is methimazole. pulmonary emboli in the right lung. He has no other med On physical examination, temperature is 38.2 'C ical problems, and he takes no medications. (100.8 'F); other vital signs are normal. Posterior pharyn- On physical examination, blood pressure is 132 17 6 mmHg, geal erythema is noted. pulse rate is 100/min, and respiration rate is 18/min. Oxygen Laboratory studies: saturation is 98% at rest breathing ambient air. Hemoglobin 13.9 g/dl (139 g/L) The patient is alert and quickly comprehends the Leukocyte count 23OO I 1tL (2.3 x r}e I L) (2O"L diagnostic implications and required therapy. He does not neutrophils, 7 2% \y mpho - require pain medication. The patient is engaged in a shared cytes, 8'1, monocytes) decision making process regarding treatment. Absolute neutrophil count 460lytL (0.+o x to!'/L) Mean corpuscular volume 92fL Which of the following is the most appropriate Platelet count 302,000/pL (302 x 10e/L) recommendation? (A) Discharge home and treat with dabigatran Which of the following is the most likely diagnosis? (B) Discharge home and treat with rivaroxaban (A) Benign ethnic neutropenia (C) Hospitalize and treat with thrombolytic therapy (B) Cyclic neutropenia (D) Hospitalize and treat with unfractionated heparin and (C) Drug induced neutropenia warfarin (D) Vitamin B,, deliciency 77

i 1 I !I

i 1 I !I Self-Assessment Test I vr i .D 'l D UI ut (D tr Item 30 A 70 year old man is hospitalizecl with a 3 day history of On physical examination, bloodpressure is 105/60 mm Hg sitting and 80/50 mm Hg standing; pulse rate is 105imin both sitting and standing. Periorbital ecchymoses, macro- 1 I UI :rcute abdominal pain and nausea and vorniting. Medical UI and family histories are noncontributory. and he takes no glossia, and jugular venous distention are present. I medications. Laboratory studies show a normal hemoglobin level, .D On physical examination. vital signs are normal. serum albumin level of 2 gldL (2O glL) , and serum creati, )t nine level of 1.5 mg/dl (133 pmol/L). 24 Hour urine albu- c la Palpation elicits mild tenderness in the upper abdomen. u,ithout hepatosplenomegaly or abdominal mass. min excretion is 3500 mg. Serum protein electrophoresis ) Laboratory studies show a hemoglobin level of shows an IgG l. spike of 1.2 mg/dl. 'l 14.5 g/dl (145 g1L), leukocyte count of50001UL (5 x 10q/L), Echocardiogram shows biventricular myocardial and platelet count ol 250.000rprL (250 x 10"iL). hypertrophy with nondilated ventricles and diastolic dys- lI

Self-Assessment Test I vr i .D 'l D UI ut (D tr Item 30 A 70 year old man is hospitalizecl with a 3 day history of On physical examination, bloodpressure is 105/60 mm Hg sitting and 80/50 mm Hg standing; pulse rate is 105imin both sitting and standing. Periorbital ecchymoses, macro- 1 I UI :rcute abdominal pain and nausea and vorniting. Medical UI and family histories are noncontributory. and he takes no glossia, and jugular venous distention are present. I medications. Laboratory studies show a normal hemoglobin level, .D On physical examination. vital signs are normal. serum albumin level of 2 gldL (2O glL) , and serum creati, )t nine level of 1.5 mg/dl (133 pmol/L). 24 Hour urine albu- c la Palpation elicits mild tenderness in the upper abdomen. u,ithout hepatosplenomegaly or abdominal mass. min excretion is 3500 mg. Serum protein electrophoresis ) Laboratory studies show a hemoglobin level of shows an IgG l. spike of 1.2 mg/dl. 'l 14.5 g/dl (145 g1L), leukocyte count of50001UL (5 x 10q/L), Echocardiogram shows biventricular myocardial and platelet count ol 250.000rprL (250 x 10"iL). hypertrophy with nondilated ventricles and diastolic dys- lI CT ol the abdonten with contrast demonstr:rtes an function. Left ventricular ejection fraction is 51%. I rcute portcl vein thrombosis. I Which of the following is the most appropriate diagnostic test to perform next? II Which of the following is the most appropriate additional diagnostic test? (A) Abdominal fat pad biopsy lI I

CT ol the abdonten with contrast demonstr:rtes an function. Left ventricular ejection fraction is 51%. I rcute portcl vein thrombosis. I Which of the following is the most appropriate diagnostic test to perform next? II Which of the following is the most appropriate additional diagnostic test? (A) Abdominal fat pad biopsy lI I (A) Antithrombin measurement (B) Endomyocardialbiopsy 1 t (B) JAK2 tyrosine kinase mutation (C) Kidney biopsy I (C) Protein C measurement (D) Tilt-table test l

(A) Antithrombin measurement (B) Endomyocardialbiopsy 1 t (B) JAK2 tyrosine kinase mutation (C) Kidney biopsy I (C) Protein C measurement (D) Tilt-table test l (D) Protein S measurement l Item 33 1 A 3O-year old man is evaluated for progressive fatigue of tr Item 31 A 74 year old man is evaluated in the hospital fbr new several months' duration. Medical history is otherwise unremarkable. He indicates having a sibling. He takes no \ onset thrombocytopenia. He was hospitalized 48 hours medications. t ago fbr management of CT angiograpl.ry confirmed pul- On physical examination, pulse rate is 114/min; other \I monary embolism. Medical history is significant fbr coro- vital signs are normal. Oxygen saturation is 98'1, breathing nary artery bypass gralt surgery 3 rteeks ago. Medications ambient air. Conjunctival rim pallor and scattered pete- I are aspirin. clopidogrel, metoprolol. lisinopril, furosentide. chiae are noted. and atorvastatinr low molecular weight heparin (LMWH) l

ago fbr management of CT angiograpl.ry confirmed pul- On physical examination, pulse rate is 114/min; other \I monary embolism. Medical history is significant fbr coro- vital signs are normal. Oxygen saturation is 98'1, breathing nary artery bypass gralt surgery 3 rteeks ago. Medications ambient air. Conjunctival rim pallor and scattered pete- I are aspirin. clopidogrel, metoprolol. lisinopril, furosentide. chiae are noted. and atorvastatinr low molecular weight heparin (LMWH) l was initiated on admission to the hospital. taboratory studies: i On physical examination. vital signs are normal. Hemoglobin 7.1gtdL(7tetL) i Other than a well l.realing sternotomy scar. the physical Leukocyte count 1200/pL (1.2 x 10e/L) examination is unremarkable. (65% neutrophils, 35'/" ! lymphocytes) t I-aboratory studies: Mean corpuscular volume 90 fL I I tiemoglobin 12 gldl. (120 g,l-) Leukocyte count 11.500/pL (11.5 x lO',tL) Platelet count 22,OOO/1tL (22xloe lL) ] Reticuloclte count 1% of erythrocytes Platelet count 90,000/pL (90 x 10"/L) (at admission: 2So,oooipL [250 x 1O'q/L]) Peripheral blood smear shows erythrocytes with nor :

tiemoglobin 12 gldl. (120 g,l-) Leukocyte count 11.500/pL (11.5 x lO',tL) Platelet count 22,OOO/1tL (22xloe lL) ] Reticuloclte count 1% of erythrocytes Platelet count 90,000/pL (90 x 10"/L) (at admission: 2So,oooipL [250 x 1O'q/L]) Peripheral blood smear shows erythrocytes with nor : Estimated glomerular >60 mLr'nrin,'1.73 m2 mal morphology and decreased platelets. A bone marrow tiltration rate biopsy shows marked hypocellularity and increased fat content without dysplastic cells. Testing for viral hepatitis Peripheral blood smear is normal. is negative. ) The.1T score is 5. corresponding to an intermediate risk Ihe sibling's test results show an HLA match. ol heparin inducted thrombocytopenia. LMWH is discon- I tinued, and a test Ibr heparin-induced antibody is ordered. 1 Which of the following is the most effective therapy? Which of the following is the most appropriate management? (A) Allogeneic hematopoietic stem cell transplantation 1

tinued, and a test Ibr heparin-induced antibody is ordered. 1 Which of the following is the most effective therapy? Which of the following is the most appropriate management? (A) Allogeneic hematopoietic stem cell transplantation 1 (A) Discontinue aspirin and clopidogrel (B) Antithymocyteglobulin ! (B) Insert an inferior vena cava filter (C) Azacytidine (C) Start rivaroxaban (D) Intravenous immune globulin (D) Start warfarin Item 34 \ A 7S-year-old man is evaluated for an elevated INR found Item 32 on routine monitoring. He is otherwise well and has no evi A 63 year old man is evaluated for easy bruising, wors- dence of bleeding. Medical history is signiflcant for atrial ening edema, and dizziness on standing over the past flbrillation. His only medication is warfarin. 9 months. Medical history is otherwise noncontributory; Laboratory studies show a normal hemoglobin level he takes no medications. and INR of 8.5. : 78

Self-Assessment Test ta 6' The patient is instructed not to take his warfarin until Item 37 F y further INR evaluation. A 68 year old woman is seen in consultation 3 weeks (u following right hemicolectomy for stage III colon cancer. E Which of the following is the most appropriate additional UI Medical history is otherwise unremarkable, and she takes UI (l, management? no medications. ut ta (A) 4-Factor prothrombin complex concentrate Physical examination is unremarkable, and the surgi cal site is healing well. (B) Factor VIIa Chemotherapy with 5 fluorouracil, leucovorin, and =(, r^ (C) Fresh frozen plasma oxaliplatin is planned. (D) Vitamin K (E) Observation Which of the following is most appropriate venous thromboembolism risk management for this patient? Item 35 (A) Calculate the Khorana score A77-year-old man is evaluated for anemia that has worsened (B) Initiate apixaban over the past year. He has fatigue and dyspnea on exertion. (C) Initiate low molecular weight heparin He has a history of poorly controlled hypertension. Medica- (D) Initiate warfarin tions are lisinopril, amlodipine, and hydrochlorothiazide. On physical examination, blood pressure is 180/100 mm Llg. The remainder of the vital signs and physical examina Item 38 tion are unremarkable. A 35-year-old man is evaluated during a routine visit for Laboratory studies: p-thalassemia intermedia. One year ago, he began requir Hemoglobin B.B g/dl (88 g/L) ing erythrocyte transfusions every 4 to 6 months fbr symp- Leukocl.te count 6800/pL (O.S x toq/L) tomatic anemia. He has no other symptoms or medical Mean corpuscular volume 88 fL problems, and his only medication is folic acid. Platelet count 41 4,0 o o I StL (41 4 x los I L) On physical examination, vital signs are normal. Fron- Reticulocyte count 1.2'1, of erythrocytes tal bossing, macrocephaly, scleral icterus, and splenomegaly Creatinine 3.2 mg/dl (283 pmol/L) are present and unchanged from previous examination. The Ferritin 150 ng/ml (ts0 pglL) remainder of the physical examrnation is unremarkable. Iron 70 pg/dl (13 pmol/L) Laboratory studies show a hemoglobin level of 7.9 g/dl Total iron binding capacity 300 pgldl (s4 pmol/L) (79 g/L),leukocyte count of 64ool1tL (6.+ x toe/L), and Stool is negative for occult blood. Peripheral blood platelet count of110,000/pL (110 x 10e/L). Iron studies were smear demonstrates normal leukocyte and erythrocyte at the higher limit of normal one and a half years ago. morphology. Ultrasound shows small echogenic kidneys bilaterally. Which of following is the most appropriate additional management? Which of the following is the most likely diagnosis? (A) Abdominalultrasonography J (A) Anemia of inflammation (B) Iron studies (B) Anemia of kidney disease (C) Prophylacticanticoagulation (C) Microangiopathic hemolytic anemia (D) Splenectomy (D) Myelodysplasticsyndrome

ta 6' The patient is instructed not to take his warfarin until Item 37 F y further INR evaluation. A 68 year old woman is seen in consultation 3 weeks (u following right hemicolectomy for stage III colon cancer. E Which of the following is the most appropriate additional UI Medical history is otherwise unremarkable, and she takes UI (l, management? no medications. ut ta (A) 4-Factor prothrombin complex concentrate Physical examination is unremarkable, and the surgi cal site is healing well. (B) Factor VIIa Chemotherapy with 5 fluorouracil, leucovorin, and =(, r^ (C) Fresh frozen plasma oxaliplatin is planned. (D) Vitamin K (E) Observation Which of the following is most appropriate venous thromboembolism risk management for this patient? Item 35 (A) Calculate the Khorana score A77-year-old man is evaluated for anemia that has worsened (B) Initiate apixaban over the past year. He has fatigue and dyspnea on exertion. (C) Initiate low molecular weight heparin He has a history of poorly controlled hypertension. Medica- (D) Initiate warfarin tions are lisinopril, amlodipine, and hydrochlorothiazide. On physical examination, blood pressure is 180/100 mm Llg. The remainder of the vital signs and physical examina Item 38 tion are unremarkable. A 35-year-old man is evaluated during a routine visit for Laboratory studies: p-thalassemia intermedia. One year ago, he began requir Hemoglobin B.B g/dl (88 g/L) ing erythrocyte transfusions every 4 to 6 months fbr symp- Leukocl.te count 6800/pL (O.S x toq/L) tomatic anemia. He has no other symptoms or medical Mean corpuscular volume 88 fL problems, and his only medication is folic acid. Platelet count 41 4,0 o o I StL (41 4 x los I L) On physical examination, vital signs are normal. Fron- Reticulocyte count 1.2'1, of erythrocytes tal bossing, macrocephaly, scleral icterus, and splenomegaly Creatinine 3.2 mg/dl (283 pmol/L) are present and unchanged from previous examination. The Ferritin 150 ng/ml (ts0 pglL) remainder of the physical examrnation is unremarkable. Iron 70 pg/dl (13 pmol/L) Laboratory studies show a hemoglobin level of 7.9 g/dl Total iron binding capacity 300 pgldl (s4 pmol/L) (79 g/L),leukocyte count of 64ool1tL (6.+ x toe/L), and Stool is negative for occult blood. Peripheral blood platelet count of110,000/pL (110 x 10e/L). Iron studies were smear demonstrates normal leukocyte and erythrocyte at the higher limit of normal one and a half years ago. morphology. Ultrasound shows small echogenic kidneys bilaterally. Which of following is the most appropriate additional management? Which of the following is the most likely diagnosis? (A) Abdominalultrasonography J (A) Anemia of inflammation (B) Iron studies (B) Anemia of kidney disease (C) Prophylacticanticoagulation (C) Microangiopathic hemolytic anemia (D) Splenectomy (D) Myelodysplasticsyndrome Item 39 Item 36 A 32 year-old man is evaluated for easy bruising. He has A 68-year-old woman is seen in consultation regarding no other medical problems; takes no medications, recre- recently diagnosed lAK2 V6l7F mutation-positive essen ational drugs, or supplements: and does not drink alcohol. tial thrombocythemia. She is asymptomatic, has no other On physical examination, vital signs are normal. Two medical problems, and takes no medications. small ecchymoses are noted on the left thigh; he also has one I On physical examination, vital signs are normal. The small bruise on the right upper arm. No petechiae are visi- spleen is palpable 2 cm below the left costal margin. ble. He has no lymphadenopathy or hepatosplenomegaly. taboratory studies show a hemoglobin level of 13.5 g/dl (135 g/L), leukocyte count of 6000/pL (o x toe/L), and Iaboratory studies: platelet count of 685,000/pL (685 x 10e/L). Hemoglobin 14.5 gtdL (14s g/L) Leukocyte count 5500/pL (s.s x roqlL) Which of the following is the most appropriate treatment? Mean corpuscular volume 8s fL Platelet count 44,ooolStL (44xloe lL) (A) Aspirin Reticulocyte count 1.2?, of erythrocl.tes (B) Hydroxyurea plus aspirin Creatinine 0.7 mg/dl (61.9 pmol/L) (C) Ruxolitinib Urinalysis Normal (D) Stem cell transplantation Peripheral blood smear is unremarkable

Item 39 Item 36 A 32 year-old man is evaluated for easy bruising. He has A 68-year-old woman is seen in consultation regarding no other medical problems; takes no medications, recre- recently diagnosed lAK2 V6l7F mutation-positive essen ational drugs, or supplements: and does not drink alcohol. tial thrombocythemia. She is asymptomatic, has no other On physical examination, vital signs are normal. Two medical problems, and takes no medications. small ecchymoses are noted on the left thigh; he also has one I On physical examination, vital signs are normal. The small bruise on the right upper arm. No petechiae are visi- spleen is palpable 2 cm below the left costal margin. ble. He has no lymphadenopathy or hepatosplenomegaly. taboratory studies show a hemoglobin level of 13.5 g/dl (135 g/L), leukocyte count of 6000/pL (o x toe/L), and Iaboratory studies: platelet count of 685,000/pL (685 x 10e/L). Hemoglobin 14.5 gtdL (14s g/L) Leukocyte count 5500/pL (s.s x roqlL) Which of the following is the most appropriate treatment? Mean corpuscular volume 8s fL Platelet count 44,ooolStL (44xloe lL) (A) Aspirin Reticulocyte count 1.2?, of erythrocl.tes (B) Hydroxyurea plus aspirin Creatinine 0.7 mg/dl (61.9 pmol/L) (C) Ruxolitinib Urinalysis Normal (D) Stem cell transplantation Peripheral blood smear is unremarkable 79

Self-Assessment Test tt .D Which of the following are the most appropriate Medical history is significant for atrial fibrillation. Medica D la diagnostic tests? tions are dabigatran and metoprolol. UI .D On physical examination, temperature is 37.0 oC (9U.6 'F). UI (A) Antinuclear antibodies and complement level l^ blood pressure is 88/60 mm Hg, pulse rate is 120, min and (B) Antiplatelet antibodies and direct antiglobulin test irregular, and respiration rate is 20/min. Oxygen satura .D (C) H lV and hepatitis C virus tion is 96'2, breathing ambient air. Cardiopulmonary exam { .D (D) Vitamin B,, and folate levels ination is normal. The abdomen is tender. Ecchvmoscs are tt noted on the left flank. Laboratory studies: Item 4O Activated partial 52 s A 72-year-old man is evaluated for progressive fatigue of thromb<iplastin tinre several months'duration. Medical history is signiflcant fbr Hemoglobin 8.4 gtdl (81 g L) carcinoma of the bladder diagnosed 6 years ago, which was Leukocyte count 11,600''pL (11.6 x 10'g L) treated with combination chemotherapy and radiation, Platelet count 428.000 gl. (a28 x 10e L) cystectomy, and creation of a urinary diversion pouch lNtl 1.6 with resection of 50 cm of ileum. He takes omeprazole for CT scan shows a large retroperitoneal hemorrhage. gastroesophageal refl ux disease. Vital signs and the remainder of the physical examina tion are noncontributory. Which of the following is the most appropriate treatment?

tt .D Which of the following are the most appropriate Medical history is significant for atrial fibrillation. Medica D la diagnostic tests? tions are dabigatran and metoprolol. UI .D On physical examination, temperature is 37.0 oC (9U.6 'F). UI (A) Antinuclear antibodies and complement level l^ blood pressure is 88/60 mm Hg, pulse rate is 120, min and (B) Antiplatelet antibodies and direct antiglobulin test irregular, and respiration rate is 20/min. Oxygen satura .D (C) H lV and hepatitis C virus tion is 96'2, breathing ambient air. Cardiopulmonary exam { .D (D) Vitamin B,, and folate levels ination is normal. The abdomen is tender. Ecchvmoscs are tt noted on the left flank. Laboratory studies: Item 4O Activated partial 52 s A 72-year-old man is evaluated for progressive fatigue of thromb<iplastin tinre several months'duration. Medical history is signiflcant fbr Hemoglobin 8.4 gtdl (81 g L) carcinoma of the bladder diagnosed 6 years ago, which was Leukocyte count 11,600''pL (11.6 x 10'g L) treated with combination chemotherapy and radiation, Platelet count 428.000 gl. (a28 x 10e L) cystectomy, and creation of a urinary diversion pouch lNtl 1.6 with resection of 50 cm of ileum. He takes omeprazole for CT scan shows a large retroperitoneal hemorrhage. gastroesophageal refl ux disease. Vital signs and the remainder of the physical examina tion are noncontributory. Which of the following is the most appropriate treatment? Iaboratory studies: (A) Anderanet alfa Hemoglobin 8.8 g/dl (BB s/L) (!) 4 tractor prothron.rbin complex concentrate Leukocyte count 4000/pL (4 x 10e/L) (C) Idirrucizumab Mean corpuscular volume 110 fL (D) Plasma Platelet count 140,000/pL (140 x l0'/L) Reticulocyte count 17, of erythrocytes Alanine aminotransferase 30 U/L Item 42 Aspartate aminotransferase 50 U/L Bilirubin A 64 year-old man is evaluated following a recent diagnosis Total 3.s mg/dl (59.9 pmol/L) of multiple myeloma. Diagnosis was based on a monoclonal Direct 0.5 mg/dl (8.6 pmol/L) spike on serum protein electrophoresis, conflrmatory bone Lactate dehydrogenase 400 u/L marrow biopsy, and two lytic lesions on bone imaging. Serum calcium level and kidney function are normal, and Peripheral blood smear is shown he has not experienced any infections. He received a herpes zoster virus vaccination at age ..,ii f, r@' 60 years, receives annual influenza vaccination, and received the COVID 19 vaccination series. &# Chemotherapy with bortezomib, lenalidomide, and dexamethasone is planned to begin in 14 days.

Iaboratory studies: (A) Anderanet alfa Hemoglobin 8.8 g/dl (BB s/L) (!) 4 tractor prothron.rbin complex concentrate Leukocyte count 4000/pL (4 x 10e/L) (C) Idirrucizumab Mean corpuscular volume 110 fL (D) Plasma Platelet count 140,000/pL (140 x l0'/L) Reticulocyte count 17, of erythrocytes Alanine aminotransferase 30 U/L Item 42 Aspartate aminotransferase 50 U/L Bilirubin A 64 year-old man is evaluated following a recent diagnosis Total 3.s mg/dl (59.9 pmol/L) of multiple myeloma. Diagnosis was based on a monoclonal Direct 0.5 mg/dl (8.6 pmol/L) spike on serum protein electrophoresis, conflrmatory bone Lactate dehydrogenase 400 u/L marrow biopsy, and two lytic lesions on bone imaging. Serum calcium level and kidney function are normal, and Peripheral blood smear is shown he has not experienced any infections. He received a herpes zoster virus vaccination at age ..,ii f, r@' 60 years, receives annual influenza vaccination, and received the COVID 19 vaccination series. &# Chemotherapy with bortezomib, lenalidomide, and dexamethasone is planned to begin in 14 days. &* & m. ir .. q4*." ..i ir'\ f, Which of the following prophylactic therapies is indicated today? t,.,-a (A) Acyclovir (B) Intravenous immune globulin ,# i. 't (C) Levofloxacin (D) Pneumococcalvaccine e& ryS* d q Item 43 Which of the following is the most likely cause of this A 48 year old woman is evaluated in the emergency patient's anemia? department for right leg swelling and pain of I week's (A) Hypothyroidism duration. She reports no recent surgery, injuries, periods (B) Immune-mediated hemolyticanemia of immobility, or long airplane flights. Medical history is unremarkable. She takes no medications. (C) Impaired vitamin B,, absorption On physical examination, vital signs are normal. BMI (D) Liver disease is 20. Oxygen saturation is 99'ln breathing ambient air. Pitting edema extends to the knee on the right leg, and the right calf is 4 cm larger than the left calf. Pulses are intact, tr Item 41 A 68-year old woman is evaluated in the emergency and capillary reflll is normal. Duplex ultrasound shows an acute thrombosis of the departrnent f<tr abdominal and back pain and hypotension. right f'emoral vein.

&* & m. ir .. q4*." ..i ir'\ f, Which of the following prophylactic therapies is indicated today? t,.,-a (A) Acyclovir (B) Intravenous immune globulin ,# i. 't (C) Levofloxacin (D) Pneumococcalvaccine e& ryS* d q Item 43 Which of the following is the most likely cause of this A 48 year old woman is evaluated in the emergency patient's anemia? department for right leg swelling and pain of I week's (A) Hypothyroidism duration. She reports no recent surgery, injuries, periods (B) Immune-mediated hemolyticanemia of immobility, or long airplane flights. Medical history is unremarkable. She takes no medications. (C) Impaired vitamin B,, absorption On physical examination, vital signs are normal. BMI (D) Liver disease is 20. Oxygen saturation is 99'ln breathing ambient air. Pitting edema extends to the knee on the right leg, and the right calf is 4 cm larger than the left calf. Pulses are intact, tr Item 41 A 68-year old woman is evaluated in the emergency and capillary reflll is normal. Duplex ultrasound shows an acute thrombosis of the departrnent f<tr abdominal and back pain and hypotension. right f'emoral vein. 80

Self-Assessment Test UI (C) Proteins C and S €, Which of the following is the most appropriate h management? (D) No additional testing c(l, (A) Anticoagulation for 3 months E ta (B) Anticoagulation for 3 months plus inferior vena cava Item 46 vt o fllter A 27 year old woman is evaluated for spontaneous bruis- t t (C) Extendedanticoagulation ing. She reports a history of heavy menses since men (D) Thrombolytictherapy arche, requiring treatment for iron deflciency anemia. Her o ur mother and maternal uncle also have a history of bleeding. She takes no medications. Item 44 On physical examination, vital signs are normal. She has ecchymoses on her extremities, but her skin texture A 70 year old man is evaluated for recently discovered and joint mobility are normal. The remainder olthe exam- erythrocytosis. Other than daytime sleepiness, he feels ination is unremarkable. well. He has a 50 pack year history of smoking and con tinues to smoke. He also has obesity, hypertension, and laboratory studies: obstructive sleep apnea. He is nonadherent with continu Activated partial 37s ous positive airway pressure (CPAP) ventilation because of thromboplastin time discomfort. His only medication is lisinopril. Hemoglobin 11.s g/dl (1ls g/L) On physical examination, vital signs are normal. BMI Leukocyte count 8000/pL (B x 1O'q/L) is 35. Oxygen saturation is 98'/. breathing ambient air. The Mean corpuscular volume 78 fL examination is unremarkable. Platelet count 140,000/pL (140 x 10,/L) Prothrombin time 13s [-aboratory studies: Factor VIII 40"1, Erythropoietin 33 mU/mL (33 U/L) Hematocrit 52'/,, The platelet function testing result is abnormal. Leukocyte count 8000/pL (S x tO"/L) Platelet count 185,000/pL (tSS x tO',/L) Which of the following is the most likely diagnosis? Genetic testing is negative for the lAK2Y6l7F mutation. (A) Ehlers-Danlossyndrome He receives brief behavioral intervention for smoking (B) Hemophilia A carrier cessation and is offered varenicline. He is referred to sleep (C) Immunethrombocytopenicpurpura medicine to discuss strategies to decrease discomfort with CPAP and to improve adherence. (D) von Willebrand disease

UI (C) Proteins C and S €, Which of the following is the most appropriate h management? (D) No additional testing c(l, (A) Anticoagulation for 3 months E ta (B) Anticoagulation for 3 months plus inferior vena cava Item 46 vt o fllter A 27 year old woman is evaluated for spontaneous bruis- t t (C) Extendedanticoagulation ing. She reports a history of heavy menses since men (D) Thrombolytictherapy arche, requiring treatment for iron deflciency anemia. Her o ur mother and maternal uncle also have a history of bleeding. She takes no medications. Item 44 On physical examination, vital signs are normal. She has ecchymoses on her extremities, but her skin texture A 70 year old man is evaluated for recently discovered and joint mobility are normal. The remainder olthe exam- erythrocytosis. Other than daytime sleepiness, he feels ination is unremarkable. well. He has a 50 pack year history of smoking and con tinues to smoke. He also has obesity, hypertension, and laboratory studies: obstructive sleep apnea. He is nonadherent with continu Activated partial 37s ous positive airway pressure (CPAP) ventilation because of thromboplastin time discomfort. His only medication is lisinopril. Hemoglobin 11.s g/dl (1ls g/L) On physical examination, vital signs are normal. BMI Leukocyte count 8000/pL (B x 1O'q/L) is 35. Oxygen saturation is 98'/. breathing ambient air. The Mean corpuscular volume 78 fL examination is unremarkable. Platelet count 140,000/pL (140 x 10,/L) Prothrombin time 13s [-aboratory studies: Factor VIII 40"1, Erythropoietin 33 mU/mL (33 U/L) Hematocrit 52'/,, The platelet function testing result is abnormal. Leukocyte count 8000/pL (S x tO"/L) Platelet count 185,000/pL (tSS x tO',/L) Which of the following is the most likely diagnosis? Genetic testing is negative for the lAK2Y6l7F mutation. (A) Ehlers-Danlossyndrome He receives brief behavioral intervention for smoking (B) Hemophilia A carrier cessation and is offered varenicline. He is referred to sleep (C) Immunethrombocytopenicpurpura medicine to discuss strategies to decrease discomfort with CPAP and to improve adherence. (D) von Willebrand disease Which of the following is the most appropriate additional Item 47 management? A 68 year old woman is evaluated in the emergency (A) Bone marrow biopsy department for swelling in her right leg of 3 days' duration. (B) Hydroxyurea She completed therapy for breast cancer I month ago. She (C) Phlebotomy takes no medications. On physical examination, vital signs are normal. She (D) Ruxolitinib has swelling of the right lower extremity from the foot to (E) No further testing or interventions the thigh with pitting edema.

Which of the following is the most appropriate additional Item 47 management? A 68 year old woman is evaluated in the emergency (A) Bone marrow biopsy department for swelling in her right leg of 3 days' duration. (B) Hydroxyurea She completed therapy for breast cancer I month ago. She (C) Phlebotomy takes no medications. On physical examination, vital signs are normal. She (D) Ruxolitinib has swelling of the right lower extremity from the foot to (E) No further testing or interventions the thigh with pitting edema. Which of the following is the most appropriate test?

Which of the following is the most appropriate additional Item 47 management? A 68 year old woman is evaluated in the emergency (A) Bone marrow biopsy department for swelling in her right leg of 3 days' duration. (B) Hydroxyurea She completed therapy for breast cancer I month ago. She (C) Phlebotomy takes no medications. On physical examination, vital signs are normal. She (D) Ruxolitinib has swelling of the right lower extremity from the foot to (E) No further testing or interventions the thigh with pitting edema. Which of the following is the most appropriate test? tr Item 45 A 35 year-old man is evaluated in the emergency depart (A) Calculate the Pulmonary Embolism Rule Out Criteria SCOTE ment fbr a 2-day history of swelling in his left leg. His (B) CT angiography mother was diagnosed r.t ith a venous thromboembolism at age 50 years. The patient can identify no provoking event. (C) D-dimermeasurement He is otheru,ise well and takes no medications. (D) Duplexultrasonography On physical examination, vital signs are normal. The entire left lower extremity is swollen. Lupus anticoagulant, anticardiolipin antibody, and Item 48 anti-B2-glycoprotein antibody testing is normal. A 77 year old man undergoes follow-up evaluation for Duplex ultrasonography confirms deep venous throm macrocytic anemia discovered last week on a routine com bosis in the left f'emoral vein. plete blood count. He was diagnosed with polycythemia vera 12 months ago. Treatment consists olthe antimetabo Which of the following should be measured now to inform lite hydroxyurea. Befbre starting hydroxyurea, vitamin 8,, immediate treatment decisions? and folate levels were normal. His only other medication is a multivitamin with folic acid. (A) Antithrombin On physical examination, vital signs and the physical (B) FactorV Leiden examination are normal.

tr Item 45 A 35 year-old man is evaluated in the emergency depart (A) Calculate the Pulmonary Embolism Rule Out Criteria SCOTE ment fbr a 2-day history of swelling in his left leg. His (B) CT angiography mother was diagnosed r.t ith a venous thromboembolism at age 50 years. The patient can identify no provoking event. (C) D-dimermeasurement He is otheru,ise well and takes no medications. (D) Duplexultrasonography On physical examination, vital signs are normal. The entire left lower extremity is swollen. Lupus anticoagulant, anticardiolipin antibody, and Item 48 anti-B2-glycoprotein antibody testing is normal. A 77 year old man undergoes follow-up evaluation for Duplex ultrasonography confirms deep venous throm macrocytic anemia discovered last week on a routine com bosis in the left f'emoral vein. plete blood count. He was diagnosed with polycythemia vera 12 months ago. Treatment consists olthe antimetabo Which of the following should be measured now to inform lite hydroxyurea. Befbre starting hydroxyurea, vitamin 8,, immediate treatment decisions? and folate levels were normal. His only other medication is a multivitamin with folic acid. (A) Antithrombin On physical examination, vital signs and the physical (B) FactorV Leiden examination are normal. 81

Self-Assessment Test vt (D laboratory studies: Which of the following is the most appropriate initial ut Haptoglobin Normal treatment for the coagulopathy? l,t Hemoglobin t2gtdL(t2ogtL) G (A) Eculizumab t^ Leukocyte count 3000/pL (3 x 10e/L) t! Mean corpuscular volume 11s fL (B) Prednisone o Platelet count 140,000/pL (140 x 10e/L) (C) Therapeutic plasma exchange Reticulocyte count 1% oferythrocytes (D) No additional treatment .D Bilirubin Normal L Lactate dehydrogenase Normal Peripheral blood smear demonstrates macrocytosis and hyperchromia. Item 50 A22 year old man is admitted to the ICU for hypotension. tr tachycardia. hypoxia, and respiratory failure requiring Which of the following is the most likely cause of the intubation. He developed a fever, tachycardia, and malaise patient's macrocytic anemia? 12 hours ago. He has B cell acute lymphoblastic leukemia that has relapsed multiple times; he received therapy with (A) Hydroxyurea chimeric antigen receptor (CAR) T cells 4 days ago. (B) Immune-mediated hemolltic anemia On physical examination, temperature is 40.0 'C (C) Paroxysmalnocturnalhemoglobinuria (104.0 "F), blood pressure is 80/50 mm Hg, pulse rate is (D) Vitamin Br2 deflciency 140/min, and respiration rate is 36/min. Oxygen saturation is 80'7, breathing high-flow oxygen. No jugular venous dis tension is noted. Diffuse crackles are heard on pulmonary

vt (D laboratory studies: Which of the following is the most appropriate initial ut Haptoglobin Normal treatment for the coagulopathy? l,t Hemoglobin t2gtdL(t2ogtL) G (A) Eculizumab t^ Leukocyte count 3000/pL (3 x 10e/L) t! Mean corpuscular volume 11s fL (B) Prednisone o Platelet count 140,000/pL (140 x 10e/L) (C) Therapeutic plasma exchange Reticulocyte count 1% oferythrocytes (D) No additional treatment .D Bilirubin Normal L Lactate dehydrogenase Normal Peripheral blood smear demonstrates macrocytosis and hyperchromia. Item 50 A22 year old man is admitted to the ICU for hypotension. tr tachycardia. hypoxia, and respiratory failure requiring Which of the following is the most likely cause of the intubation. He developed a fever, tachycardia, and malaise patient's macrocytic anemia? 12 hours ago. He has B cell acute lymphoblastic leukemia that has relapsed multiple times; he received therapy with (A) Hydroxyurea chimeric antigen receptor (CAR) T cells 4 days ago. (B) Immune-mediated hemolltic anemia On physical examination, temperature is 40.0 'C (C) Paroxysmalnocturnalhemoglobinuria (104.0 "F), blood pressure is 80/50 mm Hg, pulse rate is (D) Vitamin Br2 deflciency 140/min, and respiration rate is 36/min. Oxygen saturation is 80'7, breathing high-flow oxygen. No jugular venous dis tension is noted. Diffuse crackles are heard on pulmonary tr Item 49 A 7S-year-old man is hospitalized with sepsis secondary examination. Laboratory studies show a leukocl'te count of2400ipl (2.4 x LoelL) (reduced from 12,000i[L hz x l0qrl]) with to community-acquired pneumonia. He has been other 20'2, lymphoblasts (reduced from 80'X,) following CAR T wise well before hospital admission and was taking no cell therapy. Serum creatinine is 2 mgi dL (U6.8 [moUL)i medications. serum electrolyte and urate levels are normal. On physical examination, temperature is 39.4 'C (102.9 'F), blood pressure is 90/52 mm Hg, pulse rate is 110/min, and respiration rate is 16/min. Oxygen saturation Which of the following is the most likely diagnosis? is 92% using a nonrebreathing mask. Cardiac examination (A) Cytokine release syndrome reveals tachycardia. Bilateral basilar pulmonary crackles (B) Progressive acute lymphoblastic leukemia are noted. (C) Tumor lysis syndrome Laboratory studies: (D) Venous thromboembolism Activated partial thromboplastin 55s time D dimer 2.8 pg/ml (z.s mg/L) Item 51 Hemoglobin e.lgtdL(ergtL) A 61-year-old woman is seen in consultation regarding a Leukocyte count 14,000/pL (14 x 10e/L) recently identifled monoclonal protein. Medical history is Platelet count 55,ooo/pL (55 x 10q/L) notable for rheumatoid arthritis that has been stable for Prothrombin time 19s several years. Her only medication is methotrexate. Fibrinogen 10s mg/dl (1.0s g/L) On physical examination, vital signs and other exam- Peripheral blood smear is shown. ination flndings are normal.

tr Item 49 A 7S-year-old man is hospitalized with sepsis secondary examination. Laboratory studies show a leukocl'te count of2400ipl (2.4 x LoelL) (reduced from 12,000i[L hz x l0qrl]) with to community-acquired pneumonia. He has been other 20'2, lymphoblasts (reduced from 80'X,) following CAR T wise well before hospital admission and was taking no cell therapy. Serum creatinine is 2 mgi dL (U6.8 [moUL)i medications. serum electrolyte and urate levels are normal. On physical examination, temperature is 39.4 'C (102.9 'F), blood pressure is 90/52 mm Hg, pulse rate is 110/min, and respiration rate is 16/min. Oxygen saturation Which of the following is the most likely diagnosis? is 92% using a nonrebreathing mask. Cardiac examination (A) Cytokine release syndrome reveals tachycardia. Bilateral basilar pulmonary crackles (B) Progressive acute lymphoblastic leukemia are noted. (C) Tumor lysis syndrome Laboratory studies: (D) Venous thromboembolism Activated partial thromboplastin 55s time D dimer 2.8 pg/ml (z.s mg/L) Item 51 Hemoglobin e.lgtdL(ergtL) A 61-year-old woman is seen in consultation regarding a Leukocyte count 14,000/pL (14 x 10e/L) recently identifled monoclonal protein. Medical history is Platelet count 55,ooo/pL (55 x 10q/L) notable for rheumatoid arthritis that has been stable for Prothrombin time 19s several years. Her only medication is methotrexate. Fibrinogen 10s mg/dl (1.0s g/L) On physical examination, vital signs and other exam- Peripheral blood smear is shown. ination flndings are normal. TrJ- X{t* :& Complete blood counts, serum calcium level, and serum creatinine level are normal.

tr Item 49 A 7S-year-old man is hospitalized with sepsis secondary examination. Laboratory studies show a leukocl'te count of2400ipl (2.4 x LoelL) (reduced from 12,000i[L hz x l0qrl]) with to community-acquired pneumonia. He has been other 20'2, lymphoblasts (reduced from 80'X,) following CAR T wise well before hospital admission and was taking no cell therapy. Serum creatinine is 2 mgi dL (U6.8 [moUL)i medications. serum electrolyte and urate levels are normal. On physical examination, temperature is 39.4 'C (102.9 'F), blood pressure is 90/52 mm Hg, pulse rate is 110/min, and respiration rate is 16/min. Oxygen saturation Which of the following is the most likely diagnosis? is 92% using a nonrebreathing mask. Cardiac examination (A) Cytokine release syndrome reveals tachycardia. Bilateral basilar pulmonary crackles (B) Progressive acute lymphoblastic leukemia are noted. (C) Tumor lysis syndrome Laboratory studies: (D) Venous thromboembolism Activated partial thromboplastin 55s time D dimer 2.8 pg/ml (z.s mg/L) Item 51 Hemoglobin e.lgtdL(ergtL) A 61-year-old woman is seen in consultation regarding a Leukocyte count 14,000/pL (14 x 10e/L) recently identifled monoclonal protein. Medical history is Platelet count 55,ooo/pL (55 x 10q/L) notable for rheumatoid arthritis that has been stable for Prothrombin time 19s several years. Her only medication is methotrexate. Fibrinogen 10s mg/dl (1.0s g/L) On physical examination, vital signs and other exam- Peripheral blood smear is shown. ination flndings are normal. TrJ- X{t* :& Complete blood counts, serum calcium level, and serum creatinine level are normal. l(} #.., fl fr* Serum protein electrophoresis and immunofixation show an IgG r monoclonal protein spike of 0.8 g/dl. Serum free light chain and 24-hour urine protein electrophoresis are normal.

l(} #.., fl fr* Serum protein electrophoresis and immunofixation show an IgG r monoclonal protein spike of 0.8 g/dl. Serum free light chain and 24-hour urine protein electrophoresis are normal. #t..ohe Yz Whidr ofthe following is the most appropriate management? (A) Kidney biopsy (B) MRI of the cervical, lumbar, and thoracic spine (C) Serum p, microglobulin measurement )N :,o+ir?"i Cultures are collected. Fluid resuscitation is provided with (D) Clinicalobservation Item 52 A 78 year-old man is hospitalized with dyspnea, tr 0.9'2, saline, and broad spectrum antibiotics are initiated. light headedness, palpitations, and intcrmittent chest pain I l 82 t

Self-Assessment Test Ut o, ][ progressing over the past 7 days. Medical history is notab]e On physical examination, blood pressure is 124l64 mm F EI for atherosclerotic cardiovascular disease, hypertension, llg, pulse rate is l24imin, and respiration rate is 24i min. E q, cONr and hyperlipidemia. Medications are lisinopril, atorva- Oxygen saturation is 93% breathing ambient air. Examina statin, metoprolol, and aspirin. tion findings are otherwise noncontributory. E Ut ta On physical examination, blood pressure is 9l /53 mm Complete blood count and metabolic prolile are nor o, UI Hg and pulse rate is 107/minl oxygen saturation is 93'[, mal. Estimated glomerular filtration rate is greater than Ut breathing ambient air. Conjunctivae are pale. The remain- 60 ml-1min11.73 m2. der of the examination is normal. o vt l.atroratory studies: Which of the following is the most appropriate treatment? Haptoglobin <10 mg/dt, (100 mg/L) (A) Apixaban Hemogbbin 6.7 gtdt.(67 elL) (B) lnferior vena cava fllter placenrent Leukocyte count 9500/pL (9.5 x 10'q/L) (C) Thrombolytictherapy Platelet count 315,000/pl. (315 x 10e/L) Reticulocyte count 11'1, oferythrocltes (D) Unfractionutcd heparin A direct antiglobulin test is strongly positive for anti- IgG and weakly reactive fbr anticomplement. The blood bank reports all available units are incompatible. Item 55 A 2l-year-old woman is evaluated for fatigue and dyspnea Which of the following is the most appropriate immediate on exertion progressing over the past 4 days along with treatment? dark urine. One week ago, she completed a S-day course of azithromycin for cough and fever, which resolved with (A) Combination prednisone plus rituximab the medication. (B) Erythrocyetranslusion On physical examination, vital signs are normal. Con- (C) Emergentsplenectomy junctival icterus is noted. (D) Methylprednisokrne l^aboratory studies: (E) Rituximab Haptoglobin <10 mg/dl (100 mg/L) Hemoglobin 1O.7 gldL(ro7 gtL) Leukoc5rte count 8s00/pl (8.s x 10e/L) tr Item 53 A 59 year old woman is evaluatecl fbr fatigue, drcnching Platelet count Reticuloc5rte count 225,OOOI1tL (225 xroe IL) 9% of erythrocytes night sweats, low grade fever, and unintentional weight Direct antiglobulin test is negative for anti IgG and loss over the past 6 months. She also describes hcadache, strongly reactive for anticomplement. blurred vision. double vision, and difficulty concentrat ing. Medical history is noncontributory, and she takes no medications. Which of the following is the most appropriate management? On physical examination, vital signs are normal. Fun duscopic examination reveals dilated retinal veins, flame (A) Cold agglutinin titer hemorrhages. and papilledema. Crusted blood is present (B) Eculizumab in the nares. Generalized bulky lymphadenopathy and (C) Peripheral blood flow cytometry for CD55 and CD59 hepatosplenomegaly are present. Numerous petechiae are noted on the lower extremities. (D) Prednisone Serum protein clcctrophoresis and immunofixation show an IgM x monoclonal protein spike of 6000 mg/dl (60 g/1.). Free light chain ratio is greater than 100. Item 55 Serum viscosity is pending. A 35-year-old man is evaluated in the emergency depart- ment for a 2-day history of shortness of breath with no Whieh of the following is the most appropriate immediate cough or hemoptysis. Medical history is unremarkable, therapy? and he takes no medications. On physical examination, temperature is normal, (A) I Iydroxyurea blood pressure is 125/65 mm Hg, pulse rate is 7Olmin, (B) Intravenous immune globulin and respiration rate is 16lmin. Oxygen saturation is 99% (C) Plasmapheresis breathing ambient air. No leg swelling is present. (D) Ultrafiltration Which of the following is the most appropriate management? Item 54 (A) D-dimermeasurement A 65-year old woman is hospitalized with a diagnosis of multiple segmental pulmonary emboli in the right and (B) Duplex imaging left lungs. Medical history is otherwise unremarkable. Her (C) Pulmonary Embolism Rule Out Criteria score calcu- only medication is estrogen-medroxyprogesterone hor lation mone replacement. which has been discontinued. (D) No further evaluation

Ut o, ][ progressing over the past 7 days. Medical history is notab]e On physical examination, blood pressure is 124l64 mm F EI for atherosclerotic cardiovascular disease, hypertension, llg, pulse rate is l24imin, and respiration rate is 24i min. E q, cONr and hyperlipidemia. Medications are lisinopril, atorva- Oxygen saturation is 93% breathing ambient air. Examina statin, metoprolol, and aspirin. tion findings are otherwise noncontributory. E Ut ta On physical examination, blood pressure is 9l /53 mm Complete blood count and metabolic prolile are nor o, UI Hg and pulse rate is 107/minl oxygen saturation is 93'[, mal. Estimated glomerular filtration rate is greater than Ut breathing ambient air. Conjunctivae are pale. The remain- 60 ml-1min11.73 m2. der of the examination is normal. o vt l.atroratory studies: Which of the following is the most appropriate treatment? Haptoglobin <10 mg/dt, (100 mg/L) (A) Apixaban Hemogbbin 6.7 gtdt.(67 elL) (B) lnferior vena cava fllter placenrent Leukocyte count 9500/pL (9.5 x 10'q/L) (C) Thrombolytictherapy Platelet count 315,000/pl. (315 x 10e/L) Reticulocyte count 11'1, oferythrocltes (D) Unfractionutcd heparin A direct antiglobulin test is strongly positive for anti- IgG and weakly reactive fbr anticomplement. The blood bank reports all available units are incompatible. Item 55 A 2l-year-old woman is evaluated for fatigue and dyspnea Which of the following is the most appropriate immediate on exertion progressing over the past 4 days along with treatment? dark urine. One week ago, she completed a S-day course of azithromycin for cough and fever, which resolved with (A) Combination prednisone plus rituximab the medication. (B) Erythrocyetranslusion On physical examination, vital signs are normal. Con- (C) Emergentsplenectomy junctival icterus is noted. (D) Methylprednisokrne l^aboratory studies: (E) Rituximab Haptoglobin <10 mg/dl (100 mg/L) Hemoglobin 1O.7 gldL(ro7 gtL) Leukoc5rte count 8s00/pl (8.s x 10e/L) tr Item 53 A 59 year old woman is evaluatecl fbr fatigue, drcnching Platelet count Reticuloc5rte count 225,OOOI1tL (225 xroe IL) 9% of erythrocytes night sweats, low grade fever, and unintentional weight Direct antiglobulin test is negative for anti IgG and loss over the past 6 months. She also describes hcadache, strongly reactive for anticomplement. blurred vision. double vision, and difficulty concentrat ing. Medical history is noncontributory, and she takes no medications. Which of the following is the most appropriate management? On physical examination, vital signs are normal. Fun duscopic examination reveals dilated retinal veins, flame (A) Cold agglutinin titer hemorrhages. and papilledema. Crusted blood is present (B) Eculizumab in the nares. Generalized bulky lymphadenopathy and (C) Peripheral blood flow cytometry for CD55 and CD59 hepatosplenomegaly are present. Numerous petechiae are noted on the lower extremities. (D) Prednisone Serum protein clcctrophoresis and immunofixation show an IgM x monoclonal protein spike of 6000 mg/dl (60 g/1.). Free light chain ratio is greater than 100. Item 55 Serum viscosity is pending. A 35-year-old man is evaluated in the emergency depart- ment for a 2-day history of shortness of breath with no Whieh of the following is the most appropriate immediate cough or hemoptysis. Medical history is unremarkable, therapy? and he takes no medications. On physical examination, temperature is normal, (A) I Iydroxyurea blood pressure is 125/65 mm Hg, pulse rate is 7Olmin, (B) Intravenous immune globulin and respiration rate is 16lmin. Oxygen saturation is 99% (C) Plasmapheresis breathing ambient air. No leg swelling is present. (D) Ultrafiltration Which of the following is the most appropriate management? Item 54 (A) D-dimermeasurement A 65-year old woman is hospitalized with a diagnosis of multiple segmental pulmonary emboli in the right and (B) Duplex imaging left lungs. Medical history is otherwise unremarkable. Her (C) Pulmonary Embolism Rule Out Criteria score calcu- only medication is estrogen-medroxyprogesterone hor lation mone replacement. which has been discontinued. (D) No further evaluation 83

Self-Assessment Test r o IA vt o tr Item 57 A 38-year-old woman is evaluated 1 week before elective Platelet count Reticulocyte count Red-cell distribution 414,OOOl1rL (414 x loe/L) 1% of erythrocytes Elevated l^ cholecystectomy. Medical history is signiflcant for hemo vt globin SS sickle cell disease with approximately one or two width o vaso-occlusive pain events annually, which are managed The peripheral blood smear is shown. in the outpatient setting. Medications are folic acid and o tr oxycodone as needed during pain events. Laboratory studies show a hemoglobin level of 7.9 g/dl (7e stL). .# Which of the following is the most appropriate I perioperative management? ( (A) Postoperative simple transfusion; target hemoglobin l6 o Vt 10 g/dl (100 g/L) )s (B) Preoperativehydroxyurea (C) Preoperative exchange transfusion; target hemoglo- oo ,

r o IA vt o tr Item 57 A 38-year-old woman is evaluated 1 week before elective Platelet count Reticulocyte count Red-cell distribution 414,OOOl1rL (414 x loe/L) 1% of erythrocytes Elevated l^ cholecystectomy. Medical history is signiflcant for hemo vt globin SS sickle cell disease with approximately one or two width o vaso-occlusive pain events annually, which are managed The peripheral blood smear is shown. in the outpatient setting. Medications are folic acid and o tr oxycodone as needed during pain events. Laboratory studies show a hemoglobin level of 7.9 g/dl (7e stL). .# Which of the following is the most appropriate I perioperative management? ( (A) Postoperative simple transfusion; target hemoglobin l6 o Vt 10 g/dl (100 g/L) )s (B) Preoperativehydroxyurea (C) Preoperative exchange transfusion; target hemoglo- oo , bin S less than 30'2, (D) Preoperative simple transfusion; target hemoglobin to v A &o o00 10 g/dl (100 g/L) t tr Item 58 A 65-year-old woman is evaluated in the emergency Which ofthe following is the most appropriate management? (A) Colonoscopy department for a 2-day history of left arm swelling and moderate pain. She was recently diagnosed with osteo (B) Oral iron every other day myelitis of the flrst metatarsal on the right foot. and a left (C) Oral iron three times daily peripherally inserted central catheter (PICC) was placed (D) Hemoglobinelectrophoresis 2 weeks ago. Six weeks of intravenous antibiotic therapy is planned. She takes no other medications. On physical examination, vital signs are normal. She Item 6O has left upper extremity edema. Pulses are normal in the upper extremities, and no facial swelling is noted. The tbot A 55 year-old woman is evaluated in the emergency ulcer is clean, and granulation tissue is present. department for right leg swelling and is diagnosed with Duplex ultrasonography is performed on the left a proximal deep venous thrombosis. Medical history is upper extremity and demonstrates an acute deep venous signiflcant for end-stage kidney disease for which she is thrombosis in the brachial and axillary veins. undergoing hemodialysis. The PICC is functioning, well positioned, and not infected. Which of the following is the most appropriate Which ofthe following isthe most appropriate management? anticoagulation treatment?

bin S less than 30'2, (D) Preoperative simple transfusion; target hemoglobin to v A &o o00 10 g/dl (100 g/L) t tr Item 58 A 65-year-old woman is evaluated in the emergency Which ofthe following is the most appropriate management? (A) Colonoscopy department for a 2-day history of left arm swelling and moderate pain. She was recently diagnosed with osteo (B) Oral iron every other day myelitis of the flrst metatarsal on the right foot. and a left (C) Oral iron three times daily peripherally inserted central catheter (PICC) was placed (D) Hemoglobinelectrophoresis 2 weeks ago. Six weeks of intravenous antibiotic therapy is planned. She takes no other medications. On physical examination, vital signs are normal. She Item 6O has left upper extremity edema. Pulses are normal in the upper extremities, and no facial swelling is noted. The tbot A 55 year-old woman is evaluated in the emergency ulcer is clean, and granulation tissue is present. department for right leg swelling and is diagnosed with Duplex ultrasonography is performed on the left a proximal deep venous thrombosis. Medical history is upper extremity and demonstrates an acute deep venous signiflcant for end-stage kidney disease for which she is thrombosis in the brachial and axillary veins. undergoing hemodialysis. The PICC is functioning, well positioned, and not infected. Which of the following is the most appropriate Which ofthe following isthe most appropriate management? anticoagulation treatment? (A) Anticoagulation (A) Apixaban (B) PICC removal (B) Dabigatran (C) PICC removal and anticoagulation (C) Edoxaban (D) Thrombolytictherapy (D) Rivaroxaban

(A) Anticoagulation (A) Apixaban (B) PICC removal (B) Dabigatran (C) PICC removal and anticoagulation (C) Edoxaban (D) Thrombolytictherapy (D) Rivaroxaban Item 59 A 36 year old woman is evaluated for fatigue of 3 months' Item 61 A 42-year-old woman is evaluated in the emergency tr duration and dyspnea on exertion of 1 month's duration. department for 2-day history ol headache. dizziness, and She is l month postpartum. She takes no medications. easy bruising. Her medical history is otherwise unremark- On physical examination, vital signs are normal. Pal- able, and she takes no medications. lor is noted. No scleral icterus is seen. The remainder of the On physical examination, temperature is 38.0 "C examination is normal. (100.4 "F), blood pressure is 150/98 mm Hg, pulse rate is 104/min, and respiration rate is 16/min. A neurologic taboratory studies: examination is normal. She has no lymphadenopathy or Hemoglobin 7 .9 gldL (7e glL) (i1.1 g/dl organomegaly. Petechiae are noted on both legs. h11 g/L] in first trimester of pregnancy) laboratory studies: Leukocyte count 68OO/pL (6.8 x lOe/L) Haptoglobin Undetectable Mean corpuscular 70 fL (80 fL in first trimester of Hemoglobin B.2gldL (82 g/L) volume pregnancy) Leukocl.te count 10,200/pL (10.2 x 10e/L) 84

Self-Assessment Test ra ]{ Platelet count 8000/pL (s x tOq/t-) Iron studies showaferritinlevel of 100 ng/ml (100 pg/L), o, F IIJ Reticulocyte count 5'1, of erythrocytes an iron level of 98 pg/dl (18 pmol/L), and transferrin satura- coNl Creatinine 1.1 mgldl (Sl.ZpmollL) tion of 35%. q, Peripheral blood smear is shown. Heterozygous C282Y mutation of the HFE gene is dis- t/l |a covered on genetic testing, without H63D or S65C mutation. q, v tt UI UI t Which ofthe following isthe most appropriate management? (A) Iron chelation therapy (B) Phlebotomy (l, r (C) Serial iron studies jo t (D) No intervention

t Which ofthe following isthe most appropriate management? (A) Iron chelation therapy (B) Phlebotomy (l, r (C) Serial iron studies jo t (D) No intervention ) o o Item 64 oa A 45 year old woman is evaluated in the emergency depart-

(C) Serial iron studies jo t (D) No intervention ) o o Item 64 oa A 45 year old woman is evaluated in the emergency depart- 1 o ment for a 2 day history of right lower extremity swelling. Her only medication is a combination oral contraceptive. ; il & ot-a o On physical examination, vital signs are normal. Right lower extremity swelling is present to the knee with asso- ciated pitting edema. Which of the following is the most appropriate treatment? Duplex ultrasound demonstrates an acute deep venous (A) Caplacizumab thrombosis of the right femoral vein. (B) h.rtravenous nitroprusside The combination oral contraceptive is stopped, and alternative means ofbirth control are discussed. The patient (C) Plasma exchange plus prednisone and rituximab expresses a preference to be treated at home. (D) Platelettranslusion (E) Plasmapheresis with normal saline and 5'7, albumin Which of the following is the most appropriate treatment? replacement (A) Apixaban (B) Dabigatran tr Item 62 A 68-year old woman is evaluated in the emergency depart (C) Edoxaban (D) warfarin ment for a2-day history o[melena, with shortness of breath and light headedness that began today. Medical history is sig nificant fbr atrial fibrillation. Her only medication is warlarin. On physical examination, blood pressure is B2i 50 mm Hg, pulse rate is 126/min. and respiration rate is 20/min. Oxy Item 65 A 61 year old woman with recent SARS CoV 2 exposure tr gen saturation is 97"/,, breathing ambient air. Hemorrhage is hospitalized for hypoxemic respiratory failure; she is ofbright red blood per rectum is observed. also diagnosed with thrombocytopenia. Medical history is Laboratory studies show a hemoglobin level o16.2 g/dL otherwise unremarkable, and she takes no medications. 62 g,lL) and INR olB.2. On physical examination, temperature is 38.7 'C (101.7 'F), blood pressure is 105/80 mm IJg, pulse rate is Resuscitation is initiated with intravenous 0.9'i{, saline 114/min, and respiration rate is 28i min. Oxygen saluration is and erythrocyte transfusions. Intravenous vitamin K is 89% breathing ambient air. Cardiopulmonary examination administered. reveals bibasilar crackles. She has no petechiae or ecchymoses. Which of the following is the most appropriate additional Laboratory studies show a hemogkrbin level of'10.6 g/dl (106 glL),leukocyte count of 11,200/pL (11.2 x lo'/L), and treatment? platelet count of 23,00olpL (23 x 10"/L). (A) Andexanet alfa Cround-glass opacities consistent with SARS CoV-2 (B) 4-Factor prothrombin complex concentrate are seen on a chest radiograph, and a SARS CoV 2 test is (C) Idarucizumab positive. (D) Protamine sulfate The patient is given supplemental oxygen by nasal cannula, and treatment for severe SARS CoV 2 is initiated.

1 o ment for a 2 day history of right lower extremity swelling. Her only medication is a combination oral contraceptive. ; il & ot-a o On physical examination, vital signs are normal. Right lower extremity swelling is present to the knee with asso- ciated pitting edema. Which of the following is the most appropriate treatment? Duplex ultrasound demonstrates an acute deep venous (A) Caplacizumab thrombosis of the right femoral vein. (B) h.rtravenous nitroprusside The combination oral contraceptive is stopped, and alternative means ofbirth control are discussed. The patient (C) Plasma exchange plus prednisone and rituximab expresses a preference to be treated at home. (D) Platelettranslusion (E) Plasmapheresis with normal saline and 5'7, albumin Which of the following is the most appropriate treatment? replacement (A) Apixaban (B) Dabigatran tr Item 62 A 68-year old woman is evaluated in the emergency depart (C) Edoxaban (D) warfarin ment for a2-day history o[melena, with shortness of breath and light headedness that began today. Medical history is sig nificant fbr atrial fibrillation. Her only medication is warlarin. On physical examination, blood pressure is B2i 50 mm Hg, pulse rate is 126/min. and respiration rate is 20/min. Oxy Item 65 A 61 year old woman with recent SARS CoV 2 exposure tr gen saturation is 97"/,, breathing ambient air. Hemorrhage is hospitalized for hypoxemic respiratory failure; she is ofbright red blood per rectum is observed. also diagnosed with thrombocytopenia. Medical history is Laboratory studies show a hemoglobin level o16.2 g/dL otherwise unremarkable, and she takes no medications. 62 g,lL) and INR olB.2. On physical examination, temperature is 38.7 'C (101.7 'F), blood pressure is 105/80 mm IJg, pulse rate is Resuscitation is initiated with intravenous 0.9'i{, saline 114/min, and respiration rate is 28i min. Oxygen saluration is and erythrocyte transfusions. Intravenous vitamin K is 89% breathing ambient air. Cardiopulmonary examination administered. reveals bibasilar crackles. She has no petechiae or ecchymoses. Which of the following is the most appropriate additional Laboratory studies show a hemogkrbin level of'10.6 g/dl (106 glL),leukocyte count of 11,200/pL (11.2 x lo'/L), and treatment? platelet count of 23,00olpL (23 x 10"/L). (A) Andexanet alfa Cround-glass opacities consistent with SARS CoV-2 (B) 4-Factor prothrombin complex concentrate are seen on a chest radiograph, and a SARS CoV 2 test is (C) Idarucizumab positive. (D) Protamine sulfate The patient is given supplemental oxygen by nasal cannula, and treatment for severe SARS CoV 2 is initiated. Item 63 Which of the following is the first test to perform in A 44 year old woman is evaluated for hemochromatosis. evaluating the patient's thrombocytopenia? Her father was recently diagnosed with hemochromatosis (A) ADAMTS13 following evaluation olcryptogenic cirrhosis. She takes no (B) Antiptatelet antibodies medications. On physical examination, vital signs and examination (C) Direct antiglobulin test findings are normal. (D) Peripheral blood smear

Item 63 Which of the following is the first test to perform in A 44 year old woman is evaluated for hemochromatosis. evaluating the patient's thrombocytopenia? Her father was recently diagnosed with hemochromatosis (A) ADAMTS13 following evaluation olcryptogenic cirrhosis. She takes no (B) Antiptatelet antibodies medications. On physical examination, vital signs and examination (C) Direct antiglobulin test findings are normal. (D) Peripheral blood smear 85

Self-Assessment Test vr .D Item 66 96/min, and respiration rate is 1B/min. Oxygen saturation is 96'7, breathing ambient air. Cardiopulmonary examination ta ta A 7S-year-old woman is evaluated for fatigue and light- rcveals a clear chest and normal heart sounds. (D headedness of 72 hours' duration. She was hospitalized UI la Laboratory studies show a hemoglobin level of 10.8 g dL 2 months ago with a left femoral vein deep venous throm- (108 g L). leukocyte count of 18,000,pL (18 x lon L). and .D bosis and was discharged on apixaban. She flnished a 3-day platelet count of 112.000/pl- (112 x 10e,'L). course of trimethoprim sulfamethoxazole for an uncom- Three Iiters of intravenous 0.9')i saline and broad { o plicated urinary tract infection 1 week ago. She reports no spectrum antibiotics are administered. On hospital day 3. UI other symptoms, flank pain, joint pain, or change in the the patient is hemodynamically stable and is transferred to color of her stool. Her only medication is apixaban. the general medical ward. On physical examination, vital signs are normal. Oxy Repeat hemoglobin level is 7 .4 gl dL (tl gy1. gen saturation is 98'X, breathing ambient air. She has no ecchymoses or hematomas. Stool is negative for occult blood. Which of the following is the most appropriate treatment of this patient's anemia? taboratory studies: (A) Beginrecombinanterythropoietin Hemoglobin 9 gldL (9o glL) Leukocyte count 8000/pL (8 x 10e/L) (B) Erythrocyte transfusion to a target hemoglobin of Mean corpuscular volume 90 fL l0 g/dl (100 g/L) Platelet count 280,000/pL (280 x 10e/L) (C) Erythrocy'te translusion to a target hemoglobin ol The complete blood count 2 months ago was normal. 12 grdl (120 g,tl.) (D) No treatment Which of the following is the most appropriate next test to evaluate this patient's anemia? (A) Bone marrow aspiration and biopsy Item 69 (B) Direct antiglobulin test A 23 year-old woman is evaluated during a routine follow-up appointment. She has p-thalassemia major for (C) Reticulocyte count and peripheral blood smear which she has received approximately 15 transfusions (D) Serum iron and ferritin levels since childhood; she becomes symptomatic when the (E) Vitamin B,, and folate levels hemoglobin level is less than 7 gldL(70 g/L). Medical his- tory is otherwise noncontributory. Her only medication is folic acid. Item 57 On physical examination, vital signs are normal. Hep- A 24-year-old woman is evaluated lor pain in the right calf atosplenomegaly is present. since yesterday. She reports no shortness ofbreath, cough, laboratory studies: or chest pain. Her medical history is unremarkable, and Hemoglobin 7 .9 gldL (79 glL) she takes no medications. Iron studies On physical examination, vital signs are normal. A Ferritin 3228 ng/ml (3228 pgi L) tender venous cord is palpable in the right calf. No asym- Iron 2l21tgldL (38 pmol/L) metry in calf diameter is noted. Transferrin saturation 56% Duplex ultrasound of the right leg shows an acute thrombosis of the right lesser saphenous vein that is 7 cm in length. Which of the following is the most appropriate treatment? (A) Oral iron chelation Which of the following is the most appropriate (B) Phlebotomy management? (C) Plasmapheresis (A) Anticoagulation for 3 months (D) Transfusion at hemoglobin threshold of 6 g/dl (B) Anticoagulation for 6 weeks (60 etL) (C) Vein ligation (D) Observation with ultrasound surveillance Item 70 An 82 year old woman is hospitalized for an infected pres tr tr Item 68 A 76-year-old man is admitted to the ICU with septic shock sure ulcer. fever, and anemia. She resides in a long term care facility. Medical history is notable for a stroke 51'ears caused by a urinary tract infection (U't'l). Medical history ago. u,hich left her partially immobilized. Medications are is signiflcant for benign prostatic hyperplasia, hyperten lisinopril. aton,astatin, and aspirin. sion, and stage G3 chronic kidney disease. Before the UTI. On physical examination. she appears thin and frail. he was asymptomatic. Medications are amlodipine and 'l'emperature is 38.0 "C (100.4 'F). and other vital signs tamsulosin. are normal. She has right hemiplegia. A l0 cm x B cm On physical cxamination, temperature is 39.2 "C pressure ulcer is present on the sacrum and gluteus with (102.6 "F). blood pressure is 80/60 nrm Hg. pulse rate is visible bone.

vr .D Item 66 96/min, and respiration rate is 1B/min. Oxygen saturation is 96'7, breathing ambient air. Cardiopulmonary examination ta ta A 7S-year-old woman is evaluated for fatigue and light- rcveals a clear chest and normal heart sounds. (D headedness of 72 hours' duration. She was hospitalized UI la Laboratory studies show a hemoglobin level of 10.8 g dL 2 months ago with a left femoral vein deep venous throm- (108 g L). leukocyte count of 18,000,pL (18 x lon L). and .D bosis and was discharged on apixaban. She flnished a 3-day platelet count of 112.000/pl- (112 x 10e,'L). course of trimethoprim sulfamethoxazole for an uncom- Three Iiters of intravenous 0.9')i saline and broad { o plicated urinary tract infection 1 week ago. She reports no spectrum antibiotics are administered. On hospital day 3. UI other symptoms, flank pain, joint pain, or change in the the patient is hemodynamically stable and is transferred to color of her stool. Her only medication is apixaban. the general medical ward. On physical examination, vital signs are normal. Oxy Repeat hemoglobin level is 7 .4 gl dL (tl gy1. gen saturation is 98'X, breathing ambient air. She has no ecchymoses or hematomas. Stool is negative for occult blood. Which of the following is the most appropriate treatment of this patient's anemia? taboratory studies: (A) Beginrecombinanterythropoietin Hemoglobin 9 gldL (9o glL) Leukocyte count 8000/pL (8 x 10e/L) (B) Erythrocyte transfusion to a target hemoglobin of Mean corpuscular volume 90 fL l0 g/dl (100 g/L) Platelet count 280,000/pL (280 x 10e/L) (C) Erythrocy'te translusion to a target hemoglobin ol The complete blood count 2 months ago was normal. 12 grdl (120 g,tl.) (D) No treatment Which of the following is the most appropriate next test to evaluate this patient's anemia? (A) Bone marrow aspiration and biopsy Item 69 (B) Direct antiglobulin test A 23 year-old woman is evaluated during a routine follow-up appointment. She has p-thalassemia major for (C) Reticulocyte count and peripheral blood smear which she has received approximately 15 transfusions (D) Serum iron and ferritin levels since childhood; she becomes symptomatic when the (E) Vitamin B,, and folate levels hemoglobin level is less than 7 gldL(70 g/L). Medical his- tory is otherwise noncontributory. Her only medication is folic acid. Item 57 On physical examination, vital signs are normal. Hep- A 24-year-old woman is evaluated lor pain in the right calf atosplenomegaly is present. since yesterday. She reports no shortness ofbreath, cough, laboratory studies: or chest pain. Her medical history is unremarkable, and Hemoglobin 7 .9 gldL (79 glL) she takes no medications. Iron studies On physical examination, vital signs are normal. A Ferritin 3228 ng/ml (3228 pgi L) tender venous cord is palpable in the right calf. No asym- Iron 2l21tgldL (38 pmol/L) metry in calf diameter is noted. Transferrin saturation 56% Duplex ultrasound of the right leg shows an acute thrombosis of the right lesser saphenous vein that is 7 cm in length. Which of the following is the most appropriate treatment? (A) Oral iron chelation Which of the following is the most appropriate (B) Phlebotomy management? (C) Plasmapheresis (A) Anticoagulation for 3 months (D) Transfusion at hemoglobin threshold of 6 g/dl (B) Anticoagulation for 6 weeks (60 etL) (C) Vein ligation (D) Observation with ultrasound surveillance Item 70 An 82 year old woman is hospitalized for an infected pres tr tr Item 68 A 76-year-old man is admitted to the ICU with septic shock sure ulcer. fever, and anemia. She resides in a long term care facility. Medical history is notable for a stroke 51'ears caused by a urinary tract infection (U't'l). Medical history ago. u,hich left her partially immobilized. Medications are is signiflcant for benign prostatic hyperplasia, hyperten lisinopril. aton,astatin, and aspirin. sion, and stage G3 chronic kidney disease. Before the UTI. On physical examination. she appears thin and frail. he was asymptomatic. Medications are amlodipine and 'l'emperature is 38.0 "C (100.4 'F). and other vital signs tamsulosin. are normal. She has right hemiplegia. A l0 cm x B cm On physical cxamination, temperature is 39.2 "C pressure ulcer is present on the sacrum and gluteus with (102.6 "F). blood pressure is 80/60 nrm Hg. pulse rate is visible bone. 86

Self-Assessment Test UI o, tr Hemoglobin CONI laboratory studies: count Leukocyte 8.5 grdl (85 g/1.) 12,000/pL (12 x 10'/L) On physical examination, the patient is somnolent but arousable. Temperature is 37.7 oC (99.9'F). blood pressure is 90/50 mm IIg, pulse rate is 110/min, and respiration F E q,

count Leukocyte 8.5 grdl (85 g/1.) 12,000/pL (12 x 10'/L) On physical examination, the patient is somnolent but arousable. Temperature is 37.7 oC (99.9'F). blood pressure is 90/50 mm IIg, pulse rate is 110/min, and respiration F E q, Mean corpuscular volume 80 fL rate is 17lmin. Ecchymoses are noted on the upper and UI Ut Reticulocyte count 0.7'7, of erythrocytes lower extremities. Ascites is present. and the abdomen is q, tlUI Iron studies diffusely tender on palpation. Ferritin 800 ngi mL (8OO pgi L) laboratory studies: lron 10 pg/dl (Z gmol/I.) Activated partial thromboplastin 35 s o tt Total iron binding capacity 200 pg/dl- (36 pmol/L) time A plain radiograph of the lumbosacral spine demon D-dimer 0.7 pg/ml (0.7 mg/L) strates changes consistent with osteomyelitis. A bone cul Flemoglobin 9.1 gldL 194 glL) ture is obtained. Leukocyte count 12,000/pL (12 x 10e/L) Wound care and culture directed antibiotics are Platelet count 68,000/pL (68 x 10"/L) planned. Prothrombin time 17 s Fibrinogen 80 mg/dl- (0.8 g/L) Which of the following is the most appropriate treatment Abdominal ultrasonography den.ronstrates a nodular of this patient's anemia? appearance of the liver, moderate splenomegaly, and large (A) Erythrocyle lrilnsfusion amount <,rl ascites. Paracentesis confirms a diagnosis of (B) I.irythropoiesis stimulatingagent spontaneous bacterial peritonitis, and treatment is initi- (C) Intravenous iron replacement ated.

Mean corpuscular volume 80 fL rate is 17lmin. Ecchymoses are noted on the upper and UI Ut Reticulocyte count 0.7'7, of erythrocytes lower extremities. Ascites is present. and the abdomen is q, tlUI Iron studies diffusely tender on palpation. Ferritin 800 ngi mL (8OO pgi L) laboratory studies: lron 10 pg/dl (Z gmol/I.) Activated partial thromboplastin 35 s o tt Total iron binding capacity 200 pg/dl- (36 pmol/L) time A plain radiograph of the lumbosacral spine demon D-dimer 0.7 pg/ml (0.7 mg/L) strates changes consistent with osteomyelitis. A bone cul Flemoglobin 9.1 gldL 194 glL) ture is obtained. Leukocyte count 12,000/pL (12 x 10e/L) Wound care and culture directed antibiotics are Platelet count 68,000/pL (68 x 10"/L) planned. Prothrombin time 17 s Fibrinogen 80 mg/dl- (0.8 g/L) Which of the following is the most appropriate treatment Abdominal ultrasonography den.ronstrates a nodular of this patient's anemia? appearance of the liver, moderate splenomegaly, and large (A) Erythrocyle lrilnsfusion amount <,rl ascites. Paracentesis confirms a diagnosis of (B) I.irythropoiesis stimulatingagent spontaneous bacterial peritonitis, and treatment is initi- (C) Intravenous iron replacement ated. (D) No further treatment Which of the following tests will be most helpful in the evaluation of the patient's coagulopathy? Item 71 (A) Factor II level A 69-year old man is evaluated for increasing fatigue and dys- (B) Factor VIII level pnea of 8 months' duration. In the past 2 weeks, he has devel- (C) Factor X level oped shortness ofbreath at rest. He reports no other symptoms. (D) Thrombin time He has no other medical problems and takes no medications. On physical examination, temperature is 36.5 'C (97.7 "F), blood pressure is 140/80 mm Hg, pulse rate is 115/min, Item 73 and respiration rate is 2Oimin. Oxygen saturation is 95% A 41-year-old man is evaluated in the emergency depart- breathing ambient air. No bruising, petechia, lymphade- ment for increasing fatigue and dyspnea with exertion. He nopathy, or hepatosplenomegaly is noted. was recently diagnosed with acute uncomplicated cystitis l,aboratory studies: and started a course of nitrofurantoin 3 days ago. Hemoglobin 7.6 gtdL (76 glL) On physical examination, temperature is normal, Leukocyte count 29OOl1tL (2.9 xt}e tL) blood pressure is 106172 mm Hg, pulse rate is 104/min, (37'2, neutrophlls, 42"/,, and respiration rate is 16/min. Oxygen saturation is 98% lymphocytes, and 21"/,, breathing ambient air. He has conjunctival icterus. Other monocytes) than tachycardia, the physical examination is normal. Mean corpuscular volume 10s fL laboratory studies: Platelet count 90,000/pL (go x toe/L) Haptoglobin Undetectable Vitamin B,, and folate levels, coagulation tests, an HIV Hemoglobin 8.8 g/dl (88 g/L) test, and routine chemistries are normal. Leukocyte count 10,000/pL (10 x 10e/L) The peripheral blood smear shows macrocytic eryth Platelet count 200,000/pL (200 x 10e/L) rocytes and dysplastic neutrophils. Reticulocyte count 18% of erythrocytes Lactate dehydrogenase 458 U/L Which of the following is the most appropriate diagnostic Direct antiglobulin test is negative for IgG and C3. test to perform next? Peripheral blood smear shows microspherocytes, bite cells, (A) Bone marrow biopsy and blister cells. Nitrofurantoin is discontinued. (B) Erythropoietinlevel (C) JAK2 mutation testing Which of the following is the most appropriate additional (D) Parvovirus B19 DNA polymerase chain reaction management? (A) Erythrocyte transfusion (B) Plasma exchange tr AItem 72 55 year-old man is hospitalized for conlusion and (C) Prednisone (D) Rituximab abdominal pain. Medical histclry is notable ltrr alcohol- related liver disease. [{e takes lactulose and spironolactone. (E) Symptom monitoring

(D) No further treatment Which of the following tests will be most helpful in the evaluation of the patient's coagulopathy? Item 71 (A) Factor II level A 69-year old man is evaluated for increasing fatigue and dys- (B) Factor VIII level pnea of 8 months' duration. In the past 2 weeks, he has devel- (C) Factor X level oped shortness ofbreath at rest. He reports no other symptoms. (D) Thrombin time He has no other medical problems and takes no medications. On physical examination, temperature is 36.5 'C (97.7 "F), blood pressure is 140/80 mm Hg, pulse rate is 115/min, Item 73 and respiration rate is 2Oimin. Oxygen saturation is 95% A 41-year-old man is evaluated in the emergency depart- breathing ambient air. No bruising, petechia, lymphade- ment for increasing fatigue and dyspnea with exertion. He nopathy, or hepatosplenomegaly is noted. was recently diagnosed with acute uncomplicated cystitis l,aboratory studies: and started a course of nitrofurantoin 3 days ago. Hemoglobin 7.6 gtdL (76 glL) On physical examination, temperature is normal, Leukocyte count 29OOl1tL (2.9 xt}e tL) blood pressure is 106172 mm Hg, pulse rate is 104/min, (37'2, neutrophlls, 42"/,, and respiration rate is 16/min. Oxygen saturation is 98% lymphocytes, and 21"/,, breathing ambient air. He has conjunctival icterus. Other monocytes) than tachycardia, the physical examination is normal. Mean corpuscular volume 10s fL laboratory studies: Platelet count 90,000/pL (go x toe/L) Haptoglobin Undetectable Vitamin B,, and folate levels, coagulation tests, an HIV Hemoglobin 8.8 g/dl (88 g/L) test, and routine chemistries are normal. Leukocyte count 10,000/pL (10 x 10e/L) The peripheral blood smear shows macrocytic eryth Platelet count 200,000/pL (200 x 10e/L) rocytes and dysplastic neutrophils. Reticulocyte count 18% of erythrocytes Lactate dehydrogenase 458 U/L Which of the following is the most appropriate diagnostic Direct antiglobulin test is negative for IgG and C3. test to perform next? Peripheral blood smear shows microspherocytes, bite cells, (A) Bone marrow biopsy and blister cells. Nitrofurantoin is discontinued. (B) Erythropoietinlevel (C) JAK2 mutation testing Which of the following is the most appropriate additional (D) Parvovirus B19 DNA polymerase chain reaction management? (A) Erythrocyte transfusion (B) Plasma exchange tr AItem 72 55 year-old man is hospitalized for conlusion and (C) Prednisone (D) Rituximab abdominal pain. Medical histclry is notable ltrr alcohol- related liver disease. [{e takes lactulose and spironolactone. (E) Symptom monitoring 87

Self-Assessment Test vt (D + UI Ut .D Item 74 A 27-year old woman is diagnosed with acute femoral vein thrombosis. She has systemic lupus erythematosus Item 76 A 62 year old rvoman is l.rospitalized for an abdominal n'all tr UI hematoma at a recent surgical site and large ecchymoses UI diagnosed 3 months ago. Her only medication is hydroxy on her back and chest. She underwent elective cholecy's .D chloroquine. tectom)'7 days ago; ecchymoses appeared 2 days ago. She On physical examination, temperature is 37.9 'C has no history of blecding, and no family members have (D (100.2 'F); other vital signs are normal. A faint malar rash bleeding disorders. She is otherw,ise u'ell and takes n<r UI is noted. The left leg shows 2+ edema. medications. On physical examination, pulse rate is 110 min: other Laboratory studies: vital signs are normal. She has a 10 cm hematoma in the Activated partial 39 s right upper abdominal wall and ecchynroses on her chest thromboplastin time (aPTT) and upper back. Hemoglobin 10.8 g/dl (10s g/L) Leukocyte count 3200/pL (3.2 x 10e/L) Laboratory studies: Platelet count 302,000/pL (302 x loe/L) Activated partial 89 s (77 s after mixing studl') Prothrombin time 11 s thromboplastin time A mixing study does not correct the aPTT. Lupus anti Hemoglobin 10 g,'dl (tOO gr L)

vt (D + UI Ut .D Item 74 A 27-year old woman is diagnosed with acute femoral vein thrombosis. She has systemic lupus erythematosus Item 76 A 62 year old rvoman is l.rospitalized for an abdominal n'all tr UI hematoma at a recent surgical site and large ecchymoses UI diagnosed 3 months ago. Her only medication is hydroxy on her back and chest. She underwent elective cholecy's .D chloroquine. tectom)'7 days ago; ecchymoses appeared 2 days ago. She On physical examination, temperature is 37.9 'C has no history of blecding, and no family members have (D (100.2 'F); other vital signs are normal. A faint malar rash bleeding disorders. She is otherw,ise u'ell and takes n<r UI is noted. The left leg shows 2+ edema. medications. On physical examination, pulse rate is 110 min: other Laboratory studies: vital signs are normal. She has a 10 cm hematoma in the Activated partial 39 s right upper abdominal wall and ecchynroses on her chest thromboplastin time (aPTT) and upper back. Hemoglobin 10.8 g/dl (10s g/L) Leukocyte count 3200/pL (3.2 x 10e/L) Laboratory studies: Platelet count 302,000/pL (302 x loe/L) Activated partial 89 s (77 s after mixing studl') Prothrombin time 11 s thromboplastin time A mixing study does not correct the aPTT. Lupus anti Hemoglobin 10 g,'dl (tOO gr L) coagulant is positive, and high titer IgG B2 glycoprotein Platelet count 190.000'pl. (190 x loe L) and IgG cardiolipin antibodies are measured. Prothrombin time 11 s Fibrinogen 200 mg dL (2 g L) Factor Vlil activitv 2ni, Which of the following is the most appropriate immediate treatment? Which of the following is the most appropriate treatment? (A) Apixaban (B) Dabigatran (A) Activated factor VII (C) Fondaparinux (B) [)esmopressin (D) Low molecular weight heparin (C) Fresh frozen plasma (D) Factor VIII concentrate

coagulant is positive, and high titer IgG B2 glycoprotein Platelet count 190.000'pl. (190 x loe L) and IgG cardiolipin antibodies are measured. Prothrombin time 11 s Fibrinogen 200 mg dL (2 g L) Factor Vlil activitv 2ni, Which of the following is the most appropriate immediate treatment? Which of the following is the most appropriate treatment? (A) Apixaban (B) Dabigatran (A) Activated factor VII (C) Fondaparinux (B) [)esmopressin (D) Low molecular weight heparin (C) Fresh frozen plasma (D) Factor VIII concentrate tr Item 75 A 68 year old man is evaluated in the emergency depart llem 77 ment fbr gastrointestinal bleeding. lle reports a 3 day A79-year old woman is evaluated for shortness of breath history of dark stools and upper abdominal pain but is and fatigue. She was diagnosed with myelodysplastic syn otherwise asymptomatic. Medical history is significant for drome with 5q genetic abnormality 1 year ago. For the coronary artery disease for which he underwent drug past 2 months, she has required transfusions every 3 weeks eluting stent placement 7 months ago. Medications are because of symptomatic anemia. She has otherwise been metoprolol, atorvastatin, aspirin, and clopidogrel. well and takes no medications. On physical examination, blood pressure is l10r'80 On physical examination, pulse rate is 104/min; other mm ilg and pulse rate is 100lmin; other vital signs are vital signs are normal. Other than pale conjunctivae, the normal. Abdominal examination reveals midepigastric ten examination is unremarkable. derness. Rectal examination reveals guaiac positive stool. laboratory studies: Iaboratory studies show a hemoglobin level of 10.6 gidl Absolute neutrophil count 1430,,pL (1.4 x 10ei L) (106 gi l.) and platelet count of 150.000,pL (150 x 10"1L). Hemoglobin 6.3 grdl (63 gL) Activated partial thromboplastin time and prothrombin Leukocyte count 22OOl1tL (2.2xt}e lL) time are normal. Appropriate fluid resuscitation is initiated Platelet count 230,000/pL (230 x 100/L) along with a proton pump inhibitor. Endoscopy is planned. Cardiology consultation is obtained. A blood transfusion is scheduled for tomorrow

tr Item 75 A 68 year old man is evaluated in the emergency depart llem 77 ment fbr gastrointestinal bleeding. lle reports a 3 day A79-year old woman is evaluated for shortness of breath history of dark stools and upper abdominal pain but is and fatigue. She was diagnosed with myelodysplastic syn otherwise asymptomatic. Medical history is significant for drome with 5q genetic abnormality 1 year ago. For the coronary artery disease for which he underwent drug past 2 months, she has required transfusions every 3 weeks eluting stent placement 7 months ago. Medications are because of symptomatic anemia. She has otherwise been metoprolol, atorvastatin, aspirin, and clopidogrel. well and takes no medications. On physical examination, blood pressure is l10r'80 On physical examination, pulse rate is 104/min; other mm ilg and pulse rate is 100lmin; other vital signs are vital signs are normal. Other than pale conjunctivae, the normal. Abdominal examination reveals midepigastric ten examination is unremarkable. derness. Rectal examination reveals guaiac positive stool. laboratory studies: Iaboratory studies show a hemoglobin level of 10.6 gidl Absolute neutrophil count 1430,,pL (1.4 x 10ei L) (106 gi l.) and platelet count of 150.000,pL (150 x 10"1L). Hemoglobin 6.3 grdl (63 gL) Activated partial thromboplastin time and prothrombin Leukocyte count 22OOl1tL (2.2xt}e lL) time are normal. Appropriate fluid resuscitation is initiated Platelet count 230,000/pL (230 x 100/L) along with a proton pump inhibitor. Endoscopy is planned. Cardiology consultation is obtained. A blood transfusion is scheduled for tomorrow Which of the following is the most appropriate antiplatelet Which of the following is the most appropriate additional management? treatment? (A) Discontinue clopidogrel (A) Allogeneic hematopoietic stem cell transplantation (B) Discontinue clopidogrel and aspirin (B) Antithymocyte globulin and cyclosporine (C) Initiate platelet transf'usion (C) Intravenous immune globulin (D) No change in management (D) Lenalidomide

Which of the following is the most appropriate antiplatelet Which of the following is the most appropriate additional management? treatment? (A) Discontinue clopidogrel (A) Allogeneic hematopoietic stem cell transplantation (B) Discontinue clopidogrel and aspirin (B) Antithymocyte globulin and cyclosporine (C) Initiate platelet transf'usion (C) Intravenous immune globulin (D) No change in management (D) Lenalidomide 88