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Von Willebrand disease (VWD) is the most common bleeding disorder; it is believed to occur in approximately 1% to 2% of the population. Mucocutaneous and surgical hemorrhage in affected individuals is caused by quantitative and qualitative defects in von Willebrand factor (VWF), a large, multimeric protein that supports platelet adhesion and aggregation in the initiation of hemostasis at the time of vascular injury and functions as a carrier protein for factor VIII in the circulation. Advances in cellular and molecular biology have led to improved understanding of the pathophysiology of the disorder and development of a classification scheme that is based on quantitative and qualitative defects. Effective treatment is dependent on an accurate diagnosis using specific assays of VWF that define the various defects.