8. von Willebrand's Disease (vWD)

von Willebrand's Disease (vWD)

  • Type I - usually mild-moderate disease; heterozygous mutation - quantitative deficiency of all multimer sizes
  • Type II - usually mild-moderate; heterozygous mutation - qualitative abnormality of vWF resulting in decreased high MW multimers
  • Type III - severe vWD; absent vWF due to inheritance of two abnormal vWF alleles (homozygote or compound heterozygote)

Clinical manifestations

  • Mucocutaneous bleeding
    • epistaxis 60%
    • easy brusing, hematomas 40%
    • menorrhagia 35%
    • gingival bleeding 35%
    • GI bleeding 10%
  • Bleeding is common after trauma
    • dental extractions 50%
    • trauma/wounds 35%
    • post-partum 25%
    • post-operative 20%