Von Willebrand's 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 the Western 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 Western Cape.

PATIENTS: Twenty-two patients (14 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, 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, and there was 1 unclassifiable variant. Analysis of local factor VIII concentrates 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 for use as appropriate replacement therapy.