VONWILLEBRANDS-DISEASE IN THE WESTERN CAPE

Objective. To establish the prevalence of the various subtypes of Von Willebrand's disease (VWD) among patients with bleeding disorders in t he Western Cape and to review appropriate treatment strategies, Design . A systematic clinical and laboratory study, Setting, Haemophilia cli nics at two tertiary referral hospitals (Groote Schuur Hospital and Re d Cross War Memorial Children's Hospital) in the Western Cape.Patients . Twenty-two patients (14 females, 8 males; ages 3-55 years) were stud ied. Those studied were selected for reasons of convenience, as they w ere compliant and regular attenders at the clinics. Main outcome measu res. History of a bleeding tendency; bleeding time measurements; facto r VIII assays, von Willebrand factor (VWF) antigen assays; ristocetin co-factor assays and VWF multimer analysis. Results. Fourteen patients had typical type I VWD; 2 had type II and 5 had type III variants, an d there was 1 unclassifiable variant, Analysis of local factor VIII co ncentrates showed the presence of high-molecular-weight 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 f or use as appropriate replacement therapy.