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Von Willebrand's disease in the Western Cape
A R Bird1, M Shuttleworth, C Anderson
1Department of Haematology, Institute of Child Health, Red Cross War Memorial Children's Hospital, Cape Town.
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.