Bernard-Soulier syndrome (BSS) is a rare hereditary autosomal disorder characterized by a low platelet count, the appearance of giant platelets, prolonged bleeding time, and loss of glycoprotein I b. The patient was a 28-year-old woman referred to our hospital for tooth extraction. Under hemostatic control by the infusion of HLA-class I antigen matched platelet concentrates, 8E/8/8 were extracted. After infusion of the platelets, there was shortening of bleeding time, an increased number of platelets, and a slight increase in platelet adhesiveness. No abnormal bleeding occurred during the operation. However, ristocetin-induced platelet aggregation was unchanged. Clinically, although there was slight bleeding for several days from the wound at 8/8, it was stopped with the use of a splint and surgical pack.
