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Burkill - HemoDis

Hemorrhagic Disorders

QuestionAnswer
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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