Treatment of von Willebrand's Disease

• Published in: The New England journal of medicine Vol. 351; no. 7; pp. 683 - 694
• Main Author: Mannucci, Pier Mannuccio
• Format: Journal Article
• Language: English
• Published: Boston, MA Massachusetts Medical Society 12.08.2004
• Subjects: Aminocaproates - therapeutic use; Biological and medical sciences; Blood Component Transfusion; Deamino Arginine Vasopressin - administration & dosage; Deamino Arginine Vasopressin - adverse effects; Deamino Arginine Vasopressin - therapeutic use; Defects; Drug therapy; Factor VIII - chemistry; Factor VIII - physiology; Factor VIII - therapeutic use; Female; General aspects; Hematologic and hematopoietic diseases; Hemophilia A - physiopathology; Hemophilia A - therapy; Hemostasis - physiology; Humans; Isoantibodies; Medical sciences; Menorrhagia - etiology; Phenotype; Plasma; Platelet diseases and coagulopathies; Pregnancy; Pregnancy Complications, Hematologic - drug therapy; Tranexamic Acid - therapeutic use; von Willebrand Diseases - classification; von Willebrand Diseases - diagnosis; von Willebrand Diseases - physiopathology; von Willebrand Diseases - therapy; von Willebrand Factor - chemistry; von Willebrand Factor - physiology; von Willebrand Factor - therapeutic use; Medicine; Hemopathy; Treatment; Willebrand disease; Coagulopathy; Genetic disease
• ISSN: 0028-4793; 1533-4406
• DOI: 10.1056/NEJMra040403

Von Willebrand's disease is an inherited bleeding disorder with a prevalence as high as 1 to 2 percent in the general population, according to screening studies. However, its prevalence is deemed to be only 30 to 100 cases per million on the basis of symptomatic referrals, a prevalence that is similar to that of hemophilia A. This article discusses major advances in our understanding of the pathophysiology, molecular basis, and management of von Willebrand's disease. A review of the major advances in understanding of the pathophysiology, molecular basis, and management of von Willebrand's disease. Von Willebrand's disease is an inherited bleeding disorder with a prevalence as high as 1 to 2 percent in the general population, according to screening studies. 1 , 2 In contrast, estimates based on referral for symptoms of bleeding suggest a prevalence of 30 to 100 cases per million, which is similar to the prevalence of hemophilia A. 1 , 2 The disease was first described in 1926 by the Finnish pediatrician Erik von Willebrand, who used a rowboat to make house calls to patients with the disease in the Åland archipelago. The disease is caused by the quantitative deficiency or dysfunction of von . . .