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Biology and normal function of von Willebrand factor

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


Von Willebrand factor (VWF) is a large multimeric glycoprotein that performs two critical functions in hemostasis: it acts as a bridging molecule at sites of vascular injury for normal platelet adhesion, and under high shear conditions, it promotes platelet aggregation. VWF also acts as a carrier for factor VIII in the circulation, maintaining the normal level of factor VIII by increasing the half-life of factor VIII fivefold [1,2].

A bleeding disorder called von Willebrand disease (VWD) occurs when VWF is deficient or qualitatively abnormal. VWD is the most common of the inherited bleeding disorders, with an estimated prevalence in the general population of 1 percent by laboratory testing [3]. Symptomatic VWD is less common, approximately 0.01 percent, as estimated in hemostasis clinics [4]. Although it is primarily a congenital disorder, there are also acquired forms of the disease.

Evaluation of patients with these disorders has improved our understanding of the functions of VWF and the pathophysiology of VWD. This has taken on greater importance as the role of VWF in thrombotic diseases, such as thrombotic thrombocytopenic purpura and other forms of VWF-mediated thrombosis, is being increasingly recognized [5-11] and the use of new therapies for inhibiting VWF interactions is being explored [12].

The biology and normal functions of VWF will be reviewed here.

Diagnosis and management of disorders associated with deficient or abnormal VWF (eg, inherited VWD, acquired von Willebrand syndrome) are discussed separately:


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