Transfusion Studies in von Willebrand's Disease: Effect on Bleeding Time and Factor VIII

VON WILLEBRAND’S disease is a constitutional haemorrhagic diathesis, inherited as a simple Mendelian dominant and characterized by a prolonged bleeding time and a deficiency of Factor VIII (antihaemophilic globulin). It it sometimes referred to as angio-haemophilia, pseudo-haemophilia or vascular haemophilia. The pathogenetic mechanisms involved in this disorder have been investigated by Nilsson, Blomback and their collaborators (Nilsson, Blomback and von Francken, 1957; Nilsson, Blomback, Jorpes, Blomback and Johansson, 1957; Nilsson, Blombiick and BlombHck, 1959). They found that the infusion of Fraction 1-0, prepared by the method of BlombZck and Blombick (1956) from either normal or haemophilic plasma, corrected the bleeding time of patients with von Willebrand’s disease. Furthermore, the results of transfusion experiments suggest that the Factor-VIII deficiency of von Willebrand’s disease differs in its cause from that of haemophilia. Our previous work (Cornu, Larrieu, AIagille and Bernard, 1959; Cornu, Larrieu, Caen and Bernard, 1960, 1961) has partially confirmed the results of Nilsson and her colleagues, but we obtained an inconsistent effect on the bleeding time after the transfusion of Fraction I, prepared by Cohn‘s procedure. This paper presents the results of transfusion of fresh normal or haemophihc blood or plasma in patients with von Willebrand’s disease.