Von Willebrand disease

Revision as of 03:01, 13 October 2011 by Jswartz (talk | contribs)

Background

  • Most common inherited bleeding disorder
  • vWF has two roles:
    • 1. Acts as cofactor for platelet adhesion
    • 2. Acts as carrier protein for factor VIII extending its half life
  • vWD results from quantitative or qualitative dysfunction of Von Willebrand factor

Clinical Features

  • Skin and mucosal bleeding
    • Epistaxis, gingival bleeding, menorrhagia
  • Hemarthrosis is unusual

Diagnosis

  • Bleeding time: prolonged
  • PT: normal
  • PTT: normal-mildly prolonged
  • vWF activity level: low

Treatment

  • Avoid ASA, NSAIDs, heparin
  • Desmopressin
    • Induces release of vWF from endothelial storage sites
    • 0.3mcg/kg IV (max 20mcg) over 30min
  • Aminocaproic acid

Source

Tintinalli

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