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Hematology Thrombophilia Thrombophilia, characterized by an increased risk for VTE, can be acquired or inherited. I nherited Th rombophilia Prothrombin gene mutations and protein C deficiency, in addition to factor V Leiden mutation, account for S0% to 60% of causes of inherited thrombophilia. Factor V Leiden mutation is the most common hereditary thrombophilia in White populations. Screening asymptomatic patients for thrombophilia is not recommended even if a family history of thrombophilia is present. Diagnosis: Routine testing of patients with VTE for thrombophilic disorders is not recommended, because identification of inherited abnormalities does not alter the length of recommended anticoagulation or reliably predict the risk of recurrence. If testing is warranted, it should not be performed in the setting of an acute thrombotic event but rather weeks or months after it has occurred and when anticoagulant therapy has been discontinued, because active thrombosis and anticoagulation may alter the levels of some proteins. Acquired Thrombophil ia Causes of acquired thrombophilia include immobilization or recent surgery presence of a central venous catheter, pregnancy, oral contraceptives, kidney disease (particularly nephrotic syndrome), primary or secondary APLAS, and malignancy. Skin necrosis can occur in patients receiving warfarin without heparin because of the rapid depletion of protein C and the develop ment of a hypercoagulable state. API-AS is an autoimmune disorder in which thrombosis and fetal demise (in pregnancy) may occur. The diagnosis is based on the clinical criteria of thromboembolism or pregnancy morbidity and laboratory findings of antiphospholipid antibodies present on tvvo or more occasions at least 12 weeks apart. S?UDY TABLET Diagnosis of Antiphospholipid Syndrome Clinical Criteria Laboratory Criteria Presence of at least one of the following: Presence of at least one of the following: vascular thrombosis lupus anticoagulant )1 fetal deaths before 34th week gestation anticardiolipin antibody >3 spontaneous abortions before 1Oth week gestation anti-p2-GPl antibody DOil'T BE TRICKED . LACs are most commonly associated with thrombosis, not bleeding.
vascular thrombosis lupus anticoagulant )1 fetal deaths before 34th week gestation anticardiolipin antibody >3 spontaneous abortions before 1Oth week gestation anti-p2-GPl antibody DOil'T BE TRICKED . LACs are most commonly associated with thrombosis, not bleeding. Treatment The treatment of patients with an inherited thrombophilia and VTE is generally the same as for those patients without an inher ited thrombophilia. For persons with APLAS and a thromboembolic event, long-term warfarin therapy is indicated. IEST YOURSELF A 23 year old woman with a history of two miscarriages develops a VTE. Before beginning heparin, the aPTT is found to be prolonged. ANSWER: The most likely diagnosis is APIAS. 217