How I treat patients with von Willebrand disease

摘要: Von Willebrand disease (vWD) is a frequent inherited disorder of hemostasis that affects both sexes. Two abnormalities are characteristic the disease, which caused by deficiency or defect in multimeric glycoprotein called von factor: low platelet adhesion to injured blood vessels and defective intrinsic coagulation owing plasma levels factor VIII. There 2 main options available for treatment spontaneous bleeding episodes prophylaxis: desmopressin transfusional therapy with products. Desmopressin choice most patients type 1 vWD, who account approximately 70% 80% cases. This nontransfusional hemostatic agent raises endogenous VIII 3 5 times thereby corrects primary defects. In more severe ineffective contraindicated it usually necessary resort concentrates containing factor. Concentrates treated virucidal methods should be preferred cryoprecipitate because they equally effective perceived as safer. (Blood. 2001;97:1915-1919)