Von Willebrand's Disease and the Molecular Pathology of Hemostasis

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Abstract
Over 50 years ago, Eric von Willebrand1 described a severe hereditary bleeding disorder in an Äland Island family. The abnormality, which he referred to as "pseudo hemophilia," was characterized by a prolonged bleeding time and was associated with an autosomal inheritance pattern. Approximately 25 years later, Alexander and Goldstein2 made a major advance in the unravelling of von Willebrand's disease when they described the now well recognized dual hemostatic defect: reduced factor VIII procoagulant activity (antihemophilic factor) and a prolonged bleeding time. More recently, there has been an explosion of knowledge of the factor VIII system, and with this knowledge . . .

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