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20 Cards in this Set
- Front
- Back
palpable purpura
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hemorrhagic urticaria. assoc w Henoch-Schonlein purpura - leukocytoclastic angiitis, hypersensitivity vasculitis resulting from an immune reaction that damages vasc endothelium
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localized malformations of venules and capillaries of skin and mucous membranes
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Hereditary hemorrhagic telangectasias (Osler-weber-Rendu syndrome) - assoc w epistaxis, telangectasias on GI tract and cerebellum - often assoc w hemorrhage
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Walenstrom macroglobulinemia
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produces vascular damage from the sludging of hyperviscous blood - can also cause platelet function abnormalities
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Ricketsial and meningococcal diseases assoc w bleeding?
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incl Rocky Mt Spotted fever - involve vasc endothelium, leading to necrosis and rupture of small blood vessels
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hereditary deficiency of GPIIb-IIIa
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Glanzmann thrombasthenia - inaggregability of platelets due to def of GP11b111a, required for formation of fibrinogen bridges between adjacent platelets
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Clinical features of thrombocytopenia
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petechial cutaneous bleeding, intracranial bleeding, oozing from mucosal surfaces; dec platelet ct, prolonged bleeding time
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Idiopathic thrombocytopenic purpura
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in kids - acute, self limiting rxn to a viral ifxn; adults-chronic d/o. antiplatelet antibodies coat and damage platelets. Dx based on thrombocytopenia w nl or inc megakaryocytes
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Thrombotic thombocytopenic purpura (TTP)
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platelet derived hyaline microaggregates in small vessels, thrombocytopenia, microangiopathic hemolytic anemia --> damage RBCs = helmet cells and schistocytes
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Helmet cells
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TTP
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Clinical S/S of TTP
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neurologic abnormalities, renal insuff and fever
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Causes of TTP
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deficiency of vWF metalloprotease --> accum of very-high molecular weight multimers of vWF => platelet agg
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Bernard Soulier disease
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autosomal recessive d/o characterized by unusually large platelets and lack of platelet surface glycoprotein (GPIb-IX-V) needed for platelet adhesion
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Christmas disease
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prolonged APTT due to Factor IX def (Hemophilia B). indist from classic hemophilia
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Classic hemophilia
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Hemophilia A, factor VIII def. X-linked; prolonged APTT and nl bleeding time, platelet ct, PT and thrombin time
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Most common cause of vit K deficiency
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fat malabsorption from pancreatic of small-bowel disease
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Vitamin K def results in dec in which clotting factors?
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II, VII, IX, X; prolonged PT and APTT
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Von Willebrand Disease
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leads to failure of platelet adhesion; functional def of factor VIII occurs as a consequence of the deficit of vWF
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Dilutional coagulopathy
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due to multiple transfusions of stored blood def in platelets and factors II, V, and VIII
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MC hereditary bleeding d/o
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vW disease
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where is vWF synthesized?
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endothelial cells and megakaryocytes
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