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Introduction

Von Willebrand disease (vWD) is a bleeding disorder due to either decreased quantity or defective function of von Willebrand factor (vWF), a glycoprotein that plays a key role in hemostasis.  vWD is the most common inherited bleeding disorder, and patients typically have mucocutaneous bleeding (eg, epistaxis, gingival bleeding, heavy menstrual bleeding).

Pathophysiology and risk factors

vWF is a long, multimeric glycoprotein produced by endothelial cells and platelets and has a role in both primary and secondary hemostasis:

  • Primary hemostasis (ie, platelet adhesion and activation):  At sites of endothelial injury, vWF binds to glycoprotein (GP) 1b receptors on platelets, facilitating platelet adhesion to the exposed subendothelial collagen ( Figure 1).  vWF also contributes to hemostasis by activating platelets to bind to one other (ie, platelet aggregation), forming the primary hemostatic plug.  If primary hemostasis is impaired, patients develop easy bruising and prolonged mucocutaneous bleeding (eg, gingival bleeding, heavy menses).

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