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Hematologic lssues in Pregnancy Microangiopathy of Pregnancy Thrombophilia and Venous The thrombotic microangiopathies ol pregnancy encom- pass a spectrum of disorders with clinical f'eatures of Thromboembolism in Pregnancy microangiopathic hemolytic anemia and thrombocytope Epidem iology, Pathophysiology, a nd Risk Factors nia. The differentiating features of these disorders are The risk of \ITE in pregnancy is higher than in tl.re general noted in Table 37. population. but the absolute risk is still lou'(<l'X,). The distinction between HELLP (Hemolysis. Elevated Nevertheless. pulmonary emboli account for about 9'x, of Liver enzymes, Low Platelets) syndrome, pre eclampsia. and maternal deirtl.rs. The increased risk is a result of venous acute fatfy liver of pregnancy may be difficult but is not crucial stasis caused by uterine compression and increased venous because management for all of these is supportive and lbcused capacitance. increased circulating procoagulant factors. and on early delivery. Thrombotic thrombocyt<-rpenic purpura ar-rd decreased circulating protein S. VTE risk increases lurther in hemolytic uremic syndrome (see Platelet [)isorders) can also the postpartun.r period irfter er.rdothelirrl injury during delir' occur during pregnancy (see Table 37) and may require addi ery and an additional increase in the procoagulant factors. tional therapy. including plasma exchange or the anticomple- Factors that f'urther increase VTE risk include multiple ment agent eculizumab. births. advanced maternal age. <-rbesitll smoking. cesarean section. and varicose veins. XEY POIT{T5 1-he possibility of VTE is increased in patients u'ith . HELLP (Hemolysis, Elevated Liver enzymes, Low inherited thronrbophilias. Holl'ever. testing for these disor Platelets) syndrome, pre-eclampsia. and acute faffy liver ders in pregnancy is not routinely recommended. Deep of pregnancy manifest with microangiopathic hemoly venous thrombosis r.r.rost ollen occurs in the left lolr'er sis and, usually, thrombocytopenia; although distin extremit)'. guishing among these disorders may be difficult. the management of each is supportive and involves early delivery. Prevention Patients u,ith a history of unprovoked VTE. V'['E associated o Patients may develop thrombotic thrombocytopenic with pregnancy or oral contraceptives. or thrombophilia purpura and hemolytic uremic syndrome during preg associated with a high tl.rrombotic risk require prophylactic nancy, and these conditions are important to distin- antenatal and 6 week postpartum anticoagulation. Lou' guish from other causes of microangiopathic hemolysis moleculirruveight heparin (LMW[{) should be used in the and thrombocytopenia because more specific therapy, antenatal setting because warfarin is teratogenic and crosses such as plasma exchange, may be indicated. the placenta. and the direct oral anticoagulants have not been

Microangiopathy of Pregnancy Thrombophilia and Venous The thrombotic microangiopathies ol pregnancy encom- pass a spectrum of disorders with clinical f'eatures of Thromboembolism in Pregnancy microangiopathic hemolytic anemia and thrombocytope Epidem iology, Pathophysiology, a nd Risk Factors nia. The differentiating features of these disorders are The risk of \ITE in pregnancy is higher than in tl.re general noted in Table 37. population. but the absolute risk is still lou'(<l'X,). The distinction between HELLP (Hemolysis. Elevated Nevertheless. pulmonary emboli account for about 9'x, of Liver enzymes, Low Platelets) syndrome, pre eclampsia. and maternal deirtl.rs. The increased risk is a result of venous acute fatfy liver of pregnancy may be difficult but is not crucial stasis caused by uterine compression and increased venous because management for all of these is supportive and lbcused capacitance. increased circulating procoagulant factors. and on early delivery. Thrombotic thrombocyt<-rpenic purpura ar-rd decreased circulating protein S. VTE risk increases lurther in hemolytic uremic syndrome (see Platelet [)isorders) can also the postpartun.r period irfter er.rdothelirrl injury during delir' occur during pregnancy (see Table 37) and may require addi ery and an additional increase in the procoagulant factors. tional therapy. including plasma exchange or the anticomple- Factors that f'urther increase VTE risk include multiple ment agent eculizumab. births. advanced maternal age. <-rbesitll smoking. cesarean section. and varicose veins. XEY POIT{T5 1-he possibility of VTE is increased in patients u'ith . HELLP (Hemolysis, Elevated Liver enzymes, Low inherited thronrbophilias. Holl'ever. testing for these disor Platelets) syndrome, pre-eclampsia. and acute faffy liver ders in pregnancy is not routinely recommended. Deep of pregnancy manifest with microangiopathic hemoly venous thrombosis r.r.rost ollen occurs in the left lolr'er sis and, usually, thrombocytopenia; although distin extremit)'. guishing among these disorders may be difficult. the management of each is supportive and involves early delivery. Prevention Patients u,ith a history of unprovoked VTE. V'['E associated o Patients may develop thrombotic thrombocytopenic with pregnancy or oral contraceptives. or thrombophilia purpura and hemolytic uremic syndrome during preg associated with a high tl.rrombotic risk require prophylactic nancy, and these conditions are important to distin- antenatal and 6 week postpartum anticoagulation. Lou' guish from other causes of microangiopathic hemolysis moleculirruveight heparin (LMW[{) should be used in the and thrombocytopenia because more specific therapy, antenatal setting because warfarin is teratogenic and crosses such as plasma exchange, may be indicated. the placenta. and the direct oral anticoagulants have not been T.ABtE 37. Clinical and Laboratory Features of the Thrombotic Microangiopathies of Pregnancy Feature Pre-eclampsia HETLP AFLP TTP HUS Hypertension # -H + + Proteinu ria # # +/- + + Abdominal pain + + Jau nd ice +/- # +/- +/- Neurologic findings # Th rom bocytopenia + .H + .# # Hemolysis # + .H # Kidney disease + + +/- + #' Disseminated intravascu lar coagulation # .H Elevated aminotransferase levels + -H # +/- +/- Hypoglycemia + purpura.

T.ABtE 37. Clinical and Laboratory Features of the Thrombotic Microangiopathies of Pregnancy Feature Pre-eclampsia HETLP AFLP TTP HUS Hypertension # -H + + Proteinu ria # # +/- + + Abdominal pain + + Jau nd ice +/- # +/- +/- Neurologic findings # Th rom bocytopenia + .H + .# # Hemolysis # + .H # Kidney disease + + +/- + #' Disseminated intravascu lar coagulation # .H Elevated aminotransferase levels + -H # +/- +/- Hypoglycemia + purpura. # = always present. ++ = usually present. + = llkely to be present. +/- = may or may not be present. ) 64

Bibliography studied in pregnancy. LMW[{ crn be transitioned to unfrac t(EY P0l1{rs tionated heparin around the tinre of delivery. . Patients with a history of unprovoked venous thrombo ernbolism (VTE). VTE associated with pregnancy or oral Diagnosis contraceptives, or a strong hereditary thrombophilia Doppler compression ultrasonographl, is recluired to diirgnose require antenirtal and postpartum anticoagulation con deep venous thronrbosis. Diagnosing a puln-ronary embolism tinuing for 6 weeks after delivery. ir.r pregntrncy is challenging hcciruse ol the dcsire to limit o Unfractionated or low molecular-weight heparin r:rdiation exposure. CT pulmonary ar.rgiography leads to a should be used for anticoagulation during pregnancy in higher maternal exposure thar-r ventilation/perfnsion scan patients with venous thromboembolism because warfa ning. Pertusion only imaging can irlso be usetl to decrease rin is teratogenic and crosses the placenta, and the radiation exposure ifthe chest radiograph is uegltive. In preg direct oral anticoagulants have not been studied in nant wonlen with suspected pulr-nonarv enrbolism, D climer pregnancy; warfarin is safe to resume postpartum and testing and the Yl.lARS criteria ctrn be inrplemcr.rted to help while breastf'eeding. avoid imaging (Table 38).

studied in pregnancy. LMW[{ crn be transitioned to unfrac t(EY P0l1{rs tionated heparin around the tinre of delivery. . Patients with a history of unprovoked venous thrombo ernbolism (VTE). VTE associated with pregnancy or oral Diagnosis contraceptives, or a strong hereditary thrombophilia Doppler compression ultrasonographl, is recluired to diirgnose require antenirtal and postpartum anticoagulation con deep venous thronrbosis. Diagnosing a puln-ronary embolism tinuing for 6 weeks after delivery. ir.r pregntrncy is challenging hcciruse ol the dcsire to limit o Unfractionated or low molecular-weight heparin r:rdiation exposure. CT pulmonary ar.rgiography leads to a should be used for anticoagulation during pregnancy in higher maternal exposure thar-r ventilation/perfnsion scan patients with venous thromboembolism because warfa ning. Pertusion only imaging can irlso be usetl to decrease rin is teratogenic and crosses the placenta, and the radiation exposure ifthe chest radiograph is uegltive. In preg direct oral anticoagulants have not been studied in nant wonlen with suspected pulr-nonarv enrbolism, D climer pregnancy; warfarin is safe to resume postpartum and testing and the Yl.lARS criteria ctrn be inrplemcr.rted to help while breastf'eeding. avoid imaging (Table 38). Treatment Bibliography I-MWH is the treatment of choice fbr pregnirnt \ ronlen with VTE. Approach to Nonmalignant Leukopenia and Leukocytosis Therapy should begin with the standard recommended dose and Gibson C, Berliner N. I lou,$e evrluate an(l lreat neulropenir in aclults. Illood. 2Ol1;124:1251 8: quiz 1378. Il']\4lD: 248(,cr9381 doi:lO.l182,/bl(x)d 2014 02 then be titrated to the anti factor Xa level (O.Z 0.0 U/ml.) in the 1826 I 2 third trimester if the patient has gained a substantial amount of M.litre tr. 'l'rousslrd X. Monockrnrrl B cell lymphocytr)sis. Best Prilct Res Clin weight. Warfarin is teratogenic and should be avoided in the first I Ixenratol.2Olg;ll2:229 2118. IPN4ll),315856231 doi:lO.l016rj.ltha.2O19.06.O02

Treatment Bibliography I-MWH is the treatment of choice fbr pregnirnt \ ronlen with VTE. Approach to Nonmalignant Leukopenia and Leukocytosis Therapy should begin with the standard recommended dose and Gibson C, Berliner N. I lou,$e evrluate an(l lreat neulropenir in aclults. Illood. 2Ol1;124:1251 8: quiz 1378. Il']\4lD: 248(,cr9381 doi:lO.l182,/bl(x)d 2014 02 then be titrated to the anti factor Xa level (O.Z 0.0 U/ml.) in the 1826 I 2 third trimester if the patient has gained a substantial amount of M.litre tr. 'l'rousslrd X. Monockrnrrl B cell lymphocytr)sis. Best Prilct Res Clin weight. Warfarin is teratogenic and should be avoided in the first I Ixenratol.2Olg;ll2:229 2118. IPN4ll),315856231 doi:lO.l016rj.ltha.2O19.06.O02 trimester (although it is the preferred treirtment in some highiy Hematopoietic Stem Cells and Their Disorders thromboernbolic conditions such as mechanical heart valve. see Al l]stwrni O. Gupta N, Bakhrihah II, et irl. Clinicirl updates in adult:rcute lvnrpl]oblaslic leukenritr. Crit Rev Oncol llematol. 2016:99:l[i9 99. II']MID: MKSAP 19 Cardiovascular Medicine). but it is safe to resurne lfter 26?77876kloi: 10. l0l6 j.critre\()nc.2015. I2.007 delivery and with breastfeeding.'l'hrombolytic therapy raises the i\rber DA. Borowitz MJ, Cessnr l\4, et al. Initial diJgnostic workup of itcute risk of matemal hemorrhage and should be reserved lbr life leukemia: (luiclelinc fiom the College of American Pathologists tnd the Ar'nericiln Socieo,ot l lcmatokrg): Arch l)rthol Lab Med. 20l7rl ll:13.12 lll93. threatening situations. I-MWH ntust be interrupted at least [ ]'M ID: 28225:1031 tkri: I 0.585lt,arpa.20l6 050.1 CI'}