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Clinical presentation and diagnosis 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 inherited bleeding disorder, affecting up to 1 percent of the population as assessed by random laboratory screening, although only approximately 1 percent of these individuals are appreciably symptomatic [1]. It is characterized by mutations that lead to a decrease in the level or impairment in the action of von Willebrand factor (VWF) (table 1). Most cases are transmitted as an autosomal dominant trait that affects males and females equally [2]. There are also acquired forms of VWD that are caused by several different pathophysiologic mechanisms. (See "Classification and pathophysiology of von Willebrand disease" and "Biology and normal function of von Willebrand factor".)

The clinical presentation and diagnosis of VWD will be reviewed here. Separate topic reviews discuss the pathophysiology and treatment of VWD, acquired von Willebrand syndrome (aVWS), and the functions of VWF:

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

Treatment of VWD – (See "Treatment of von Willebrand disease".)

aVWS (pathophysiology, diagnosis, and treatment) – (See "Acquired von Willebrand syndrome".)

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Literature review current through: Nov 2017. | This topic last updated: Mar 20, 2017.
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