Advances in the treatment of von Willebrand disease.
Von Willebrand disease (VWD) is the most commonly inherited bleeding disorder, caused by inheritance of a quantitative or qualitative abnormality of von Willebrand factor (VWF). While the majority of patients with VWD are successfully treated with adjunctive therapies or with the synthetic vasopressin analog desmopressin acetate (DDAVP), a subset of patients requires replacement therapy. In the past, cryoprecipitate was the mainstay of therapy; however, it was associated with seroconversion to hepatitis B (HBV), hepatitis C (HCV), and the human immunodeficiency virus (HIV) in treated individuals. With the advent of virucidal methodology and, more recently, nucleic acid testing (NAT) of plasma fractions, the plasma-derived concentrates containing VWF are now considered the therapeutic standard of care.
Hambleton
J
J
Hemostasis and Thrombosis Service, University of California, San Francisco, CA 94143-1270, USA.
D016913:Blood Component Transfusion; D003894:Deamino Arginine Vasopressin / Q000627:therapeutic use; D006801:Humans; D014842:von Willebrand Diseases / Q000175:diagnosis / Q000628:therapy*; D014841:von Willebrand Factor / Q000235:genetics / Q000378:metabolism / Q000627:therapeutic use
D014841:von Willebrand Factor; D003894:Deamino Arginine Vasopressin