Presence of factor VIII-related antigen in blood platelets of patients with Von Willebrand's disease

Abstract

VON WILLEBRAND'S disease (VWD) is an autosomally inherited disorder characterised by low factor VIII activity (antihaemophilic factor, AHF), prolonged bleeding time, reduced retention of platelets in a glass bead column and abnormal distocetin-induced platelet aggregation. The prolonged bleeding time in VWD has been attributed to the absence of a plasma factor, the von Willebrand factor (VWF), as shown by a correction of the bleeding time after infusion of normal and haemophilic plasmas1. Addition of purified factor VIII in vitro specifically corrects the abnormal platelet retention and ristocetin aggregation in VWD2–5, whereas transfusion of similar material into dogs with VWD also corrects the prolonged bleeding time (B.N.B., W. J. Dodds, J. A. van Mourik, J.J.S and W. P. Webster, unpublished). This led to the suggestion that factor VIII is closely related if not identical to VWF, although dissociation of factor VIII procoagulant activity from factor VIII-related antigen (F VIII-RA) is observed in certain conditions6,7. The plasma concentration of F VIII-RA is usually reduced in VWD suggesting a reduced synthesis of factor VIII (VWF)8. In contrast to this we now report the presence of normal concentrations of F VIII-RA in platelets of patients with VWD. This F VIII-RA supported aggregation induced by ristocetin in a washed platelet system9, a property of factor VIII which has been attributed to VWF activity.