title: Management of von Willebrand disease: a survey on current clinical practice from the haemophilia centres of North America.
abstract: The optimal treatment of patients with von Willebrand's disease (vWD) remains to be defined. Moreover, it has not been firmly established which, if any, commonly measured parameters of von Willebrand factor (vWF) protein in the plasma are useful in guiding treatment. To better understand what guidelines physicians follow in the management of vWD, we surveyed 194 North American physicians who are members of the Hemophilia Research Society. Ninety-nine per cent of responding physicians depend on factor VIII (FVIII):C, vWF:RCo activity and vWF:AG to diagnose vWD, while only 49% use the bleeding time. The minimal goals of treatment for patients undergoing major surgery/trauma or central nervous system haemorrhage were FVIII:C and vWF:RCo activity greater than 80% while levels of more than 50% for minor surgery and dental extractions were considered adequate. Treatment of vWD was based on the type of vWD with type 1 patients being treated most often with desmopressin acetate (DDAVP) alone, types 2A and 2B patients with a combination of DDAVP and a vWF-containing FVIII product, type 3 patients with vWF-containing concentrate. Viral infections, including human immunodeficiency virus, hepatitis A, B and C viruses, and parvovirus have been seen in vWD and the efficacy of viral attenuation processes is a major criterion for the selection of treatment by physicians. Based on this survey, prospective studies need to be designed to address the clinical efficacy, safety and predictive value of laboratory monitoring of patients with vWD.
authors.affiliation: Newark Beth Israel Medical Center, Newark, NJ, USA. acohen@sbhcs.com
authors.forename: A J
authors.initials: AJ
authors.lastname: Cohen
authors.forename: C M
authors.initials: CM
authors.lastname: Kessler
authors.forename: B M
authors.initials: BM
authors.lastname: Ewenstein
mesh_terms: D000554:Ambulatory Care Facilities; D003625:Data Collection; D003894:Deamino Arginine Vasopressin / Q000627:therapeutic use; D019468:Disease Management; D005169:Factor VIII / Q000009:adverse effects / Q000627:therapeutic use; D006470:Hemorrhage / Q000209:etiology; D006801:Humans; D007239:Infections / Q000635:transmission; D009656:North America; D010314:Partial Thromboplastin Time; D012958:Societies, Scientific; D014842:von Willebrand Diseases / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy*; D014841:von Willebrand Factor / Q000009:adverse effects / Q000627:therapeutic use
chemical_list: D014841:von Willebrand Factor; D005169:Factor VIII; D003894:Deamino Arginine Vasopressin