Classic von Willebrand disease (vWD) - USMLE Forums
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Old 07-05-2011
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Blood Classic von Willebrand disease (vWD)

  1. Epidemiology
    1. Autosomal dominant disorder
    2. Most common hereditary coagulation disorder
  2. Pathogenesis
    • Decreased vWF and factor VIII:C activity
  3. Clinical findings in vWD
    1. Menorrhagia, epistaxis, easy bruisability
    2. Association with angiodysplasia of the right colon (see Chapter 17)
  4. Laboratory findings in vWD
    1. Increased PTT and a normal PT
    2. Increased bleeding time
      • Due a platelet adhesion defect
    3. Abnormal ristocetin cofactor assay
    4. Decreased vWF antigen
    5. Decreased VIII:Ag and VIII:C activity
  5. Treatment of vWD
    1. Desmopressin acetate (increases vWF and VIII:C activity)
    2. Oral contraceptive (estrogen has a similar action as desmopressin)
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