How I treat patients with von Willebrand disease.
Von Willebrand disease (vWD) is a frequent inherited disorder of hemostasis that affects both sexes. Two abnormalities are characteristic of the disease, which is caused by a deficiency or a defect in the multimeric glycoprotein called von Willebrand factor: low platelet adhesion to injured blood vessels and defective intrinsic coagulation owing to low plasma levels of factor VIII. There are 2 main options available for the treatment of spontaneous bleeding episodes and for bleeding prophylaxis: desmopressin and transfusional therapy with plasma products. Desmopressin is the treatment of choice for most patients with type 1 vWD, who account for approximately 70% to 80% of cases. This nontransfusional hemostatic agent raises endogenous factor VIII and von Willebrand factor 3 to 5 times and thereby corrects both the intrinsic coagulation and the primary hemostasis defects. In patients with the more severe type 3 and in most patients with type 2 disease, desmopressin is ineffective or is contraindicated and it is usually necessary to resort to plasma concentrates containing both factor VIII and von Willebrand factor. Concentrates treated with virucidal methods should be preferred to cryoprecipitate because they are equally effective and are perceived as safer. (Blood. 2001;97:1915-1919)
Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Milan, Italy. piermannuccio.mannucci@unimi.it
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D016063:Blood Loss, Surgical / Q000517:prevention & control; D003131:Combined Modality Therapy; D000075202:Contraindications; D003894:Deamino Arginine Vasopressin / Q000008:administration & dosage / Q000009:adverse effects / Q000494:pharmacology / Q000627:therapeutic use*; D004730:Endothelium, Vascular / Q000187:drug effects; D005169:Factor VIII / Q000302:isolation & purification / Q000627:therapeutic use*; D005260:Female; D006470:Hemorrhage / Q000209:etiology / Q000517:prevention & control / Q000628:therapy; D006801:Humans; D007518:Isoantibodies / Q000276:immunology; D008297:Male; D009203:Myocardial Infarction / Q000139:chemically induced; D019106:Postoperative Hemorrhage / Q000517:prevention & control; D006473:Postpartum Hemorrhage / Q000517:prevention & control; D011247:Pregnancy; D011250:Pregnancy Complications, Hematologic / Q000188:drug therapy; D017483:Receptors, Vasopressin / Q000187:drug effects; D012449:Safety; D020521:Stroke / Q000139:chemically induced; D013921:Thrombocytopenia / Q000139:chemically induced; D065227:Transfusion Reaction; D014777:Virus Diseases / Q000517:prevention & control / Q000635:transmission; D014842:von Willebrand Diseases / Q000097:blood / Q000145:classification / Q000188:drug therapy / Q000628:therapy*; D014841:von Willebrand Factor / Q000235:genetics / Q000276:immunology / Q000302:isolation & purification / Q000627:therapeutic use*
D007518:Isoantibodies; D017483:Receptors, Vasopressin; D014841:von Willebrand Factor; D005169:Factor VIII; D003894:Deamino Arginine Vasopressin