CURRENT Essentials of Critical Care.pdf

Chapter 4 Bleeding & Transfusions 41■■■Coagulopathy, InheritedEssentials of Diagnosis• Excessive or prolonged bleeding from punctures, incisions, GItract, mucosal membranes, joints, retroperitoneal space, othersites• History of lifelong abnormal bleeding or family history of bleedingdisorders• Abnormally prolonged coagulation time (prothrombin time [PT]or activated partial thromboplastin time [aPTT])• Common: von Willebrand disease (autosomal dominant deficiencyof von Willebrand factor with qualitative platelet dysfunctionand prolonged aPTT due to factor VIII deficiency); hemophiliaA (sex-linked, variably dysfunctional factor VIII);hemophilia B (sex-linked deficiency of active factor IX).• Rare: deficiency of factors II, V, VII, X, XI, XIII, fibrinogen• Inheritance of gene coding abnormal coagulation factor or insufficientproduction of a factor; X-linked or autosomalDifferential Diagnosis• Acquired coagulopathy• Thrombocytopenia or qualitative platelet disorder, vitamin C deficiency• Abnormal aPTT without risk of bleeding (lupus anticoagulant)Treatment• Establish etiology• Treat if active bleeding, high risk for bleeding, anticipated invasiveprocedure (lumbar puncture, central venous catheter, surgery)• von Willebrand disease: desmopressin (intravenous or intranasal),cryoprecipitate• VIII deficiency: desmopressin for mild bleeding, purified or recombinantfactor VIII• IX deficiency: Purified or recombinant factor IX• Fresh frozen plasma contains factors VIII, IX, most other factors,but should not be used unless others not available■ PearlA woman with a hereditary coagulopathy almost always has von Willebranddisease.ReferenceBolton-Maggs PH et al: Haemophilias A and B. Lancet 2003;361:1801. [PMID:12781551]