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Epidemiology

Authoring team

As described in the classification section, there are three types of von Willebrand disease. Considering epidemiology from a UK perspective:

  • Type 1 occurs in about 1% of the population; this is the most common type of von Willebrand disease accounting for about 75% of people with von Willebrand disease (1)
  • Type 2 accounts for 20-30% of those with von Willebrand disease (2)
  • Severe (type 3) VWD accounts for only 5% of cases and has an overall prevalence of 1 to 3 per million of the general population, but is relatively more common in countries or regions where consanguineous relationships are accepted. (3)

Reference

  1. Castaman G, Federici AB, Rodeghiero F, et al. Von Willebrand's disease in the year 2003: towards the complete identification of gene defects for correct diagnosis and treatment. Haematologica. 2003;88:94-108.
  2. Sadler JE, Budde U, Eikenboom JC, et al. Update on the pathophysiology and classification of von Willebrand disease: a report of the subcommittee on von Willebrand factor. J Thromb Haemost. 2006 Oct;4(10):2103-14.
  3. Srivastava A, Rodeghiero F. Epidemiology of von Willebrand disease in developing countries. Sem Thromb Hemost. 2005 Nov;31(5):569-76.

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