The usual cause of von Willebrand disease is an inherited 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.
Rarely, von Willebrand disease can develop later in life in people who didn't inherit an abnormal gene from a parent. This is known as acquired von Willebrand disease. The exact cause of this type of von Willebrand disease isn't clear. It may be an autoimmune disease, or it may be linked to a slow thyroid gland (hypothyroidism) or to certain medications, such as the anti-seizure medication valproic acid (Depakene) or the antibiotic ciprofloxacin (Cipro).
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- The diagnosis, evaluation, and management of von Willebrand disease. National Heart, Lung, and Blood Institute. http://www.nhlbi.nih.gov/guidelines/vwd/index.htm. Accessed Sept. 11, 2013.
- Longo DL, et al. Harrison's Online. 18th ed. New York, N.Y.: The McGraw-Hill Companies; 2012. http://www.accessmedicine.com/resourceTOC.aspx?resourceID=4. Accessed Sept. 11, 2013.
- Berntorp E. Von Willebrand disease. Pediatric Blood Cancer. 2013;60:s34.
- Rick ME. Clinical presentation and diagnosis of von Willebrand disease. http://www.uptodate.com/home/index. Accessed Sept. 11, 2013.
- Rick ME. Treatment of von Willebrand disease. http://www.uptodate.com/home/index. Accessed Sept. 11, 2013.
- Facts about von Willebrand disease. U.S. Centers for Disease Control and Prevention. http://www.cdc.gov/ncbddd/vwd/facts.html. Accessed Sept. 11, 2013.
- Von Willebrand disease. The Merck Manuals: The Merck Manual for Healthcare Professionals. http://www.merckmanuals.com/professional/hematology_and_oncology/thrombocytopenia_and_platelet_dysfunction/von_willebrand_disease.html. Accessed Sept. 11, 2013.
- What is von Willebrand disease? National Heart, Lung, and Blood Institute. http://www.nhlbi.nih.gov/health/health-topics/topics/vwd/printall-index.html. Accessed Sept. 11, 2013.
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