Von Willebrand Disease

Von Willebrand Disease is a topic covered in the 5-Minute Emergency Consult.

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  • Coagulopathy caused by deficiency or dysfunction of von Willebrand factor (vWF)
  • vWF functions:
    • Mediates platelet–endothelial cell adhesion
    • Carrier protein for factor VIII
  • Prevalence as high as 1–2% in the general population
  • Genetics:
    • Most cases inherited—multiple genetic defects identified
    • Type 1—quantitative defect of vWF:
      • 70% of cases
      • Autosomal dominant
      • vWF deficiency results from decreased synthesis and increased clearance of protein.
      • Manifestation ranges from asymptomatic to moderate bleeding.
    • Type 2—qualitative defect of vWF:
      • 10–15% of cases
      • Divided into types 2A, 2B, 2M, 2N—all are autosomal dominant except 2N which is autosomal recessive.
      • Decrease in intermediate and high molecular-weight multimer
      • 2N—decreased binding to factor VIII
      • Leads to decreased levels of VIII and thus more serious coagulopathy
    • Type 3—absent or severe deficiency in amount of vWF:
      • Rare disease—1 per million cases
      • Autosomal recessive
      • Severe coagulopathy
    • vWD genetically associated with sickle cell disease, hemophilia A, factor XII deficiency, hereditary hemorrhagic telangiectasia, and thrombocytopenia


  • In addition to genetic causes, acquired forms exist.
  • Multiple mechanisms:
    • vWF antibody production
    • Decreased synthesis
    • Proteolysis
    • Increased clearance from binding to tumor cells
  • Seen in association with the following:
    • Malignancy:
      • Wilms tumor
      • Multiple myeloma
      • Chronic lymphocytic leukemia
      • Non-Hodgkin lymphoma
      • Chronic myelogenous leukemia
      • Waldenstrom macroglobulinemia
      • Monoclonal gammopathy of uncertain significance
    • Immunologic:
      • Systematic lupus erythematosus
      • Rheumatoid arthritis
    • Medication induced:
      • Valproic acid
      • Ciprofloxacin
      • Hetastarch
      • Griseofulvin
    • Miscellaneous:
      • Hypothyroidism
      • Uremia
      • Hemoglobinopathies
      • Cirrhosis
      • Congenital heart disease
      • Disseminated intravascular coagulation

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