Audit of clinical management of von Willebrand disease during 1997 at a single institution and review of treatment patterns between 1980 and 1997

In 1997 the UK Haemophilia Centre Directors Organization published the guid elines for diagnosis and management of von Willebrand disease (vWD). The gu idelines 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 ineffe ctive in type 3 vWD. If patients are unresponsive to DDAVP or if it is cont raindicated, the treatment of choice is clotting factor concentrates (CFC). In the light of these guidelines, we audited our practice for 1997. Furthe rmore, we undertook a retrospective review of the changing patterns of trea tment of vWD between 1980 and 1997. During 1997, 10 patients with vWD recei ved DDAVP and another 30 patients were treated with CFC (a total of 1.2 mil lion IU): Haemate P (Centeon, Germany) and/or 8Y (BPL, Elstree, UK). Few pa tients 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 Will ebrand factor - where DDAVP was considered suboptimal for adequate haemosta sis. However, this assumption was made without a preliminary test dose to a ssess 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 concl usion, the audit illustrated a general good adherence to guidelines but it highlighted the need for a DDAVP test before using CFC.