Von Willebrand disease

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Causes

By Mayo Clinic staff

The cause of von Willebrand disease is a hereditary defect in the gene that controls von Willebrand factor, a protein that plays a key role in your blood clotting process. When von Willebrand factor is scarce — or not functioning properly because of structural abnormalities — small blood cells called platelets cannot stick together properly, nor can they attach themselves normally to the blood vessel walls when an injury has occurred. The result is interference with the clotting process, and uncontrolled bleeding may persist.

Von Willebrand factor carries an additional substance, called factor VIII, that helps stimulate clotting. Many people with von Willebrand disease also have low levels of factor VIII.

References
  1. Von Willebrand disease. National Heart, Lung, and Blood Institute. http://www.nhlbi.nih.gov/health/dci/Diseases/vWD/vWD_WhatIs.html. Accessed Dec. 17, 2010.
  2. Rick ME. Clinical presentation and diagnosis of von Willebrand disease. http://www.uptodate.com/home/index. Accessed Dec. 17, 2010.
  3. Rick ME. Treatment of von Willebrand disease. http://www.uptodate.com/home/index. Accessed Dec. 12, 2008. Accessed Dec. 17, 2010.
  4. Noel P (expert opinion). Mayo Clinic, Rochester, Minn. Jan. 14, 2011.
  5. Rosenow EC (expert opinion). Mayo Clinic, Rochester, Minn. Dec. 20, 2010.
DS00903 Feb. 5, 2011

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