Coagulation Disorders Flashcards
X-linked recessive hemorrhagic disease due to mutation in F8 or F9 gene causing coag factor deficiency
Hemophilia (A/classic - factor VIII, B - factor IX)
Typical coagulation test findings in hemophilia
Prolonged aPTT
Normal PT, bleeding time, platelet count
Mainstay of hemophilia treatment in response to bleeding or as prophylaxis
Factor replacement therapy
Non-transfusion therapy for mild or moderate hemophilia A
DDAVP (causes transient rise in Factor VIII and VWF, but NOT Factor IX)
Major complication of hemophilia treatment
Formation of alloantibodies to FVIII and FIX (“inhibitor”)
Major infectious cause and 2nd leading cause of death in hemophilia patients exposed to older clotting factor concentrates
Hepatitis C
Rare bleeding disorder but highly prevalent among Ashkenazi and Iraqi Jewish population
Factor XI deficiency
Fat soluble vitamin that is a cofactor for the formation of gamma-carboxyglutamic acid residues on coagulation proteins
Vitamin K
Clinicopathologic syndrome characterized by widespread intravscular fibrin formation in response to excessive blood protease activity that overcomes the natural anticoagulant mechanisms
Disseminated intravascular coagulation (DIC)
Severe form of DIC resulting from thrombosis of extensive areas of the skin
Purpura fulminans
Central mechanism of DIC
Uncontrolled thrombin generation by exposure of blood to pathologic levels of tissue factor
Most sensitive test for DIC
Elevated level of FDP (fibrin degradation product)
Most common and earliest finding in Vitamin K deficient patients
Prolonged prothrombin time
Most effective therapy to correct hemostasis in patients with liver failure
FFP transfusion (5-10mL/kg; each bag contains ~200mL)
