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20 Cards in this Set

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