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%