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8 Cards in this Set

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describe the dual role of von Willebrand factor(vWF) in hemostasis
primary hemostasis:
secondary hemostasis:
primary hemostasis:vWF binds to GPIb on the surface of platelets to mediate adhesion, especially in arterioles

secondary hemostasis:vWF is the protective carrier of factor 8
17yof with a 4 year history of heavy menses, her father had increased bleeding with a tooth extraction as a teenager. mother and susan's only sister have no bleeding history; factor 8 and vWF antigen levels are both decreased at 36% and 25% respectively; platelet count is 221,000/microliter; a multimer analysis shows absent large and intermedidiate multimers; ristocetein cofactor activity is 9%
type?
type 2a vWD
31yof with history of heavy menses and increased bleedind with her two obstetric deliveries; no family history is available; factor 8 level is 52%, vWF antigen is 48%, platelet aggregation with ristocetin is increased and her platelet count is 47,000/microliters; genetic analysis of her vWF gene was NORMAL
type?
platelet type (pseudo-vWD)
similar to the findings of type 2b but the platelets aggregate if exposed to cryoprecipitate, while in 2b they do not
10yom develops epistaxis in the waiting room after a minor confrontation eith elaine; his mother has a history of mildly increased bleeding with deliveries; his factor 8 level is 28% and vWF antigen is 30%; his platelet count is 276,000/microliter; multimer analysis shows all multimer to be slightly decreased
type?
type 1
35yom whose mother was diagnosed with vWD during her pregnancy after a complete blood count showed an abnormality; his factor ristocetin cofactor activity is 28% , vWF antigen is 70%, and he shows an increased response to ristocetin on platelrt aggregation study; the largest multimer of vWF are significantly decreased on his multimer analysis; platelet count is 60,000/microliter
type?
type 2b
profound thrombocytopenia and bleeding upon exposure to DDAVP
12yof demostrated increased bruising but is otherwise asymptomatic; her father had significant bleeding with an appendectomy and required cryoprecipitate intraoperatively; her factor 8 level is 21% an vWF antigen is 26%; platelet count is 207,000/microliter; multimer analysis shows mild decrease in low and intermediate-sized forms; but it is difficult to assess if larger multimers afre decreased
type?
type 1
treatment of type 1 is DDAVP or cryoprecipitate
5yof with multiple bruises, purpura and petechiae sitting quietly in the waiting room; factor 8 level is 6%, vWF antigen level is zero; her multimer analysis reveal no multimers; platelet count is 185,000/microliters
type?
type 3
treatment for type 3 is cryoprecipitate or factor 8 concentrate(brand that contains vWF)
True or False.
increased bleeding diathesis in myelodysplastic syndromes is due to both quantitative and a myriad of qualitative defects.
True