Von Willebrand’s Disease with Normal Factor VIII Activity in a Homozygote

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Abstract
An atypical form of von Willebrand’s disease is described in a patient apparently homozygous for an abnormal autosomal gene. The characteristics of the disease were a low factor VIII antigen level, reduced platelet adhesiveness and prolonged bleeding time, but factor VIII activity was normal and platelet aggregation by ristocetin was nearly normal. Further studies showed that the patient had a high-molecular-weight factor VIII compound, most of which, however, seemed to be somewhat smaller (less aggregated) than that in normal plasma.

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