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Burkill - HemoDis
Hemorrhagic Disorders
| Question | Answer |
|---|---|
| Disorders of Primary Hemostasis | defect in platelet plug formation petechial hemorrhages epistaxis gum bleeding GI bleeds prolonged bleeding time BT PT, PTT are normal |
| Disorders of Primary Hemostasis - vascular lesions | small vessel dysfunction - no labs but prolonged BT simply purpura, easy bruising senile purpura scurvy henonch-schonlein purpura hereditary hemorrhagic telangiectasia connective tissue disorder Waldenstroms amyloidosis rickettsia, meningitis |
| Scurvy | gingival hemorrhage hemorrhagic perifollicular hyperkeratotic papules with twisted, corkscrew hair |
| henonch-schonlein purpura | hemorrhagic urticaria, fever, arthralgias, GI and renal |
| hereditary hemorrhagic telangiectasia | Osler-Weber-Rendau localized malformations of venules and capillaries complicated by hemorrhage |
| Connective tissue disorders | Ehlers-Danlos abnormal collagen and elastin |
| Disorders of Primary Hemostasis - platelet disorders (quantitative platelet dysfunction) | Thrombocytopenia irridiation acute leukemia myelophthisis aplastic anemia splenic sequestration multiple transfusions DIC ITP TTP |
| Thrombocytopenia | petechial cutaneous bleeding oozing from mucous surfaces decreased platelet count prolonged BT |
| DIC | depletion of platelets secondary to HIV or LUPUS |
| ITP | thrombocytopenia immune reaction to viral illness anti-platelet antibodies help spleen remove platelets Diagnose - thrombocytopenia, increased megakaryocytes, no splenomegaly |
| TTP | platelet derived hyaline microaggregates in small vessels thrombocytopenia MAHA HELMETS SCHISTOCYTES neuro and renal insuff ADAMTS13 affecting vWF, creating multimers |
| Disorders of Primary Hemostasis - functional abnormalities (qualitative platelet dysfunction) | associated with mucocutaneous bleeding and associated with prolonged bleeding time bernard soulier von willegrand |
| defects of platelet adhesion | von Willebrand Bernard Soulier |
| defect of platelet aggregation | aspirin induced acetylation and inactivation of COX-1 and COX-2 so there is little TXA2 Glanzmann thrombasthenia - deficiency of GPIIb-IIIa so no fibrinogen bridges between platelets |
| Disorders of Secondary Hemostasis | Hemophilia A, B, and Vit K deficiency deficiency of plasma clotting factors bleeding from larger vessels - eccymoses, hematomas, hemarthroses abnormalities in PT, PTT, and thrombin time |
| Classic Hemophilia, Hemophilia A | most common hemophilia flassic factor VIII deficiency X-linked disorder bleeding into muscules subcutaneous tissues and joints prolonged PPT and APPT normal Bleeding time, platelet count, PT, thrombin time correct PTT with addition of normal plasma |
| Christmas Disease (hemophilia B) | factor IX deficiency less common same symptoms and x-linked |
| Vit K deficiency | often complicated by fat malabsorption decreased factor II, VII, IX, X causing increased PT, PTT |
| Von Willebrand Disease | most common hereditary bleeding disorder vWF carries factor VIII but vWF is long multimer vWF also mediates platelet-surface adhesion through GPI-b-IX-V primary bleeding disorder with increased Bleeding time functional deficiency of VIII |
| DIC | coagulative consumption of platelets and II, V, VIII and fibrinogen split products thrombosis hemorrhage MAHA schistocytes fibrin and fibrinogen split products activation of intrinsic factor pathway |
| coagulopathy of liver disease | as liver damage progresses PT, PTT and Thrombin Time are increased |
| dilutional coagulopathy | multiple transfusions causes persistant bleeding |
Created by:
burkill
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