Von Willebrand’s Disease

• Published in: The New England journal of medicine Vol. 375; no. 21; pp. 2067 - 2080
• Main Authors: Leebeek, Frank W.G, Eikenboom, Jeroen C.J
• Format: Journal Article
• Language: English
• Published: United States Massachusetts Medical Society 24.11.2016
• Subjects: Age Factors; Algorithms; Bleeding; Blood diseases; Deamino Arginine Vasopressin - therapeutic use; Female; Genetic disorders; Hematology; Hemorrhage - etiology; Humans; Male; Menorrhagia - drug therapy; Menorrhagia - etiology; Mucosa; Mutation; Surgery; Trauma; von Willebrand Diseases - classification; von Willebrand Diseases - diagnosis; von Willebrand Diseases - drug therapy; von Willebrand Diseases - genetics; von Willebrand Factor - genetics; von Willebrand Factor - physiology; von Willebrand Factor - therapeutic use; Von Willebrand's disease; Netherlands
• ISSN: 0028-4793; 1533-4406
• DOI: 10.1056/NEJMra1601561

Von Willebrand's disease is the most common inherited bleeding disorder and is generally transmitted as an autosomal dominant trait. It is mainly associated with mucosal bleeding and excessive bleeding after trauma or surgery. A variety of effective treatments are available. Von Willebrand’s disease is an inherited bleeding disorder characterized by defective platelet adhesion and aggregation. The disorder was first described in 1926 by Erik von Willebrand, who recognized that it differed from hemophilia and named it “hereditary pseudohemophilia.” 1 The factor in plasma that corrects the disease was not identified until many years later and was called von Willebrand factor. It binds to collagen at sites of vascular injury, mediates platelet adhesion and aggregation, and serves as a carrier protein for coagulation factor VIII (Figure 1). 3 Von Willebrand factor also has other functions and may be involved in such processes as . . .