title: Audit of clinical management of von Willebrand disease during 1997 at a single institution and review of treatment patterns between 1980 and 1997.
abstract: In 1997 the UK Haemophilia Centre Directors Organization published the guidelines for diagnosis and management of von Willebrand disease (vWD). The guidelines stated that desmopressin (DDAVP) should be used in type 1 and type 2N vWD, that it might be useful in other types 2 vWD and that it is ineffective in type 3 vWD. If patients are unresponsive to DDAVP or if it is contraindicated, the treatment of choice is clotting factor concentrates (CFC). In the light of these guidelines, we audited our practice for 1997. Furthermore, we undertook a retrospective review of the changing patterns of treatment of vWD between 1980 and 1997. During 1997, 10 patients with vWD received DDAVP and another 30 patients were treated with CFC (a total of 1.2 million IU): Haemate P (Centeon, Germany) and/or 8Y (BPL, Elstree, UK). Few patients had clear contraindications to DDAVP, but several patients with type 1 and 2 vWD received CFC on the basis of age or reduced levels of von Willebrand factor - where DDAVP was considered suboptimal for adequate haemostasis. However, this assumption was made without a preliminary test dose to assess the response to DDAVP. The analysis of treatment of vWD for the past 17 years showed that cryoprecipitate was discontinued from use in the early 1990s and that both DDAVP and CFC usage has been on the increase. In conclusion, the audit illustrated a general good adherence to guidelines but it highlighted the need for a DDAVP test before using CFC.
authors.affiliation: Haemophilia Centre and Haemostasis Unit, Royal Free and University College Medical School of University College London, UK.
authors.forename: I C
authors.initials: IC
authors.lastname: Nitu-Whalley
authors.forename: A H
authors.initials: AH
authors.lastname: Miners
authors.forename: C A
authors.initials: CA
authors.lastname: Lee
mesh_terms: D001779:Blood Coagulation Factors / Q000627:therapeutic use; D003894:Deamino Arginine Vasopressin / Q000627:therapeutic use; D006801:Humans; D008485:Medical Audit; D017410:Practice Guidelines as Topic; D006113:United Kingdom; D014842:von Willebrand Diseases / Q000628:therapy*; D014841:von Willebrand Factor / Q000627:therapeutic use
chemical_list: D001779:Blood Coagulation Factors; D014841:von Willebrand Factor; D003894:Deamino Arginine Vasopressin