Week 9 PDF - Hemorrhagic Disorders

9 HEMORRHAGIC DISORDERS DISORDERS OF THROMBOSIS A. PRIMARY 1. Antithrombin-III Deficiency Principal inhibitor of thrombin is deficient 2. Protein C and S Deficiency Protein C and S are Vit. K dependent glycoproteins that inactivates factors Va and VIIIa 3. Fibrinolytic System Disorders Quantitative...

9 HEMORRHAGIC DISORDERS DISORDERS OF THROMBOSIS A. PRIMARY 1. Antithrombin-III Deficiency Principal inhibitor of thrombin is deficient 2. Protein C and S Deficiency Protein C and S are Vit. K dependent glycoproteins that inactivates factors Va and VIIIa 3. Fibrinolytic System Disorders Quantitative and functional abnormalities of plasminogen Deficiency in Factor XII 2. Dysfibrinogenemia An abnormal functional fibrinogen molecule is produced and is resistant to fibrinolysis B. SECONDARY 1. Lupus anticoagulant immunoglobulin directed against the phospholipid Associated with thrombosis due to prostacyclin inhibition Prolonged APTT that does not correct with normal plasma substitution 2. Hemostatic Protein Abnormalities POST-OPERATIVE STATES Due to the release of tissue thromboplastin which initiates coagulation Malignancy Release of coagulating factors by neoplastic cells Pregnancy Placenta is rich in tissue thromboplastin HEMORRHAGIC DISORDERS A. INTRINSIC PATHWAY DISORDERS 1. Factor XI Deficiency (Hemophilia C) AKA ROSENTHAL SYNDROME Mild bleeding Autosomal More than half of the cases have been described in Ashkenazi Jews 2. Factor VIII: C Deficiency (Hemophilia A) Sex-linked Factor VIII:C is complexed with Factor VIII:vWF 3. Factor IX Deficiency (Hemophilia B) AKA Christmas Disease Sex-linked HEMORRHAGIC DISORDERS A. INTRINSIC PATHWAY DISORDERS 1. Factor XI Deficiency (Hemophilia C) AKA ROSENTHAL SYNDROME Mild bleeding Autosomal More than half of the cases have been described in Ashkenazi Jews 2. Factor VIII: C Deficiency (Hemophilia A) Sex-linked Factor VIII:C is complexed with Factor VIII:vWF 3. Factor IX Deficiency (Hemophilia B) AKA Christmas Disease Sex-linked VON WILLEBRAND'S DISEASE Defects of both Factor VIII:C and vWF PROLONGED APTT, PROLONGED BT, NORMAL PT MOST FREQUENTLY ENCOUNTERED HEREDITARY COAGULOPATHY CBC, APTT & PT ARE RECOMMENDED FOR INITIAL WORKUP HEMORRHAGIC DISORDERS B. Extrinsic and Common Pathway Disorders 1. Factor VII Deficiency Abnormal PT, Normal APTT and thrombin time 2. Factor X (Stuart-Prower Factor) Deficiency abnormal APTT and PT, normal TCT 3. Factor V Deficiency (Owen's Disease) abnormal APTT and PT, normal TCT 4. Factor II (Prothrombin) Deficiency abnormal APTT and PT, normal TCT 5. Factor I Deficiency Abnormal APTT, PT and TCT AFIBRINOGENEMIA complete absence HYPOFIBRINOGENEMIA low levels/ decreased (