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Van Willebrand's disease in the Western Cape
Abstract
Objective. To establish the prevalence of the various subtypes of Van Willebrand's disease (VWD) among patients with bleeding disorders in the Westem Cape and to review appropriate treatment strategies.
Design. A systematic clinical and laboratory study.
Setting. Haemophilia clinics at two tertiary referral hospitals (Groote Schuur Hospital and Red Cross War Memorial Children's Hospital) in the Westem Cape. Patients. Twenty-two patients (t 4 females, 8 males; ages 3 - 55 years) were studied. Those studied were selected for reasons of convenience, as they were compliant and regular attenders at the clinics.
Main outcome measures. History of a bleeding tendency; bleeding time measurements; factor VIII assays, van Willebrand factor (VWF) antigen assays; ristocetin co-factor assays and VWF multimer analysis. Results. Fourteen patients had typical type I VWD; 2 had type 11 and 5 had type III variants, and there was 1 unclassifiable variant. Analysis of local factor VIII concentrates showed the presence of high-molecularweight VWF multimers.
Conclusion. The results are similar to patterns reported elsewhere in the world. Locally produced factor VIII concentrates, unlike a number of commercially produced concentrates, contain sufficient multimers for use as appropriate replaceinent therapy.
