Diagnosis and treatment of von Willebrand disease.
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.
Cox Gill
Joan
J
Department of Pediatrics, Medical College of Wisconsin, 8701 Watertown Plank Road, Milwaukee, WI 53226, USA. coxgillj@mcw.edu
D006801:Humans; D014842:von Willebrand Diseases / Q000175:diagnosis* / Q000235:genetics / Q000503:physiopathology / Q000628:therapy*; D014841:von Willebrand Factor / Q000032:analysis / Q000235:genetics
D014841:von Willebrand Factor