Abstract
von Willebrand's disease is an autosomal dominant bleeding disorder, characterised in most patients by an extended bleeding-time, low factor-VIII activity, and defective platelet adhesion to glass beads. Patients with von Willebrand's disease have low levels of factor-VIII-related antigen and defective platelet aggregation in response to the antibiotic ristocetin. Factor-VIII activity, factor-VIII-related antigen, and ristocetin-induced platelet aggregation were studied in 12 patients with von Willebrand's disease. Ristocetin-induced platelet aggregation was absent or grossly impaired in all patients. 6 of the 12 patients had detectable levels of factor-VIII-related antigen, ranging from 15 to 80% of normal. However, the presence of this antigenic material did not produce ristocetin-induced platelet aggregation, indicating that the antigen was qualitatively abnormal. The findings suggest that in von Willebrand's disease impairment of ristocetin aggregation may be due to either a low concentration of or a qualitative defect in factor-VIII-related antigen.
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