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Treatment of von Willebrand disease

Margaret E Rick, MD
Section Editor
Lawrence LK Leung, MD
Deputy Editor
Jennifer S Tirnauer, MD


Von Willebrand disease (VWD) is the most common of the inherited bleeding disorders, with a prevalence of approximately 1 percent when random laboratory screening is performed. Although VWD is common, only a fraction of patients come to medical attention because of bleeding symptoms and are diagnosed as having VWD. This low incidence of bleeding is due to the mild nature of the disease in many patients, and to the lack of bleeding challenges and/or lack of recognition of minor excessive bleeding (eg, heavy menstrual bleeding) in others. Thus, symptomatic VWD seen at hemostasis centers has a prevalence of only approximately 0.01 percent.

VWD is characterized by mutations that lead to an impairment in the synthesis or action of von Willebrand factor (VWF). There are also acquired forms of VWD that are caused by several different pathophysiologic mechanisms. VWF plays an important role in primary hemostasis by binding to both platelets and endothelial components, forming an adhesive bridge between platelets and vascular subendothelial structures and between adjacent platelets at sites of endothelial injury. VWF also contributes to fibrin clot formation by acting as a carrier protein for factor VIII, which has a greatly shortened half-life unless it is bound to VWF.

The treatment of VWD will be reviewed here. Discussions of the function of VWF, and the pathophysiology, classification, clinical presentation, and diagnosis of VWD are presented separately.

(See "Biology and normal function of von Willebrand factor".)

(See "Classification and pathophysiology of von Willebrand disease".)


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Literature review current through: Sep 2016. | This topic last updated: Dec 9, 2015.
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