Daily Bolus of LR: von Willebrand’s Disease

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January 26, 2012 by dailybolusoflr

Von Willebrand’s Disease

  • Is the most common congenital bleeding disorder
  • Autosomal dominant in most forms, but there does exist a rare autosomal recessive form
  • Disease manifestations are:
    • related primarily to deficiency in von Willebrand’s factor which is essential for platelet adhesion to the subendothelium and for platelet-to-platelet adhesion
    • related secondarily to the fact that von Willebrand factor is also a carrier for Factor VIII
  • Bleeding is typically mucosal (epistaxis), cutaneous, menstrual and less commonly GI.  Hemarthroses are rare
  • The most commonly ordered test to diagnose this is a bleeding time, which is prolonged. 
  • Treatment for mild to moderate disease is the use of despmopressin, which increases autologous FVIII and VWF in the plasma
  • For patients with severe disease, the use of Factor VII has benefit and when not available, FFP can be used

Ref: Rosen’s 7th ed and Expert Rev. Hematol. 4(1), 95–106 (2011)

 

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