A DENTAL SURGERY OF 2 RADICULAR CYST IN A PATIENT WITH BERNARD-SOULIER SYNDROME USING PLATELET CONCENTRATES PREPARED BY A CELL SEPARATOR SPECTRA (COBE)

Bernard-Soulier syndrome is a congential bleeding diathesis characterized by an autosomal recessive inheritance and appearance of dysfunctional giant platelet in circulation. We describe here a successful treatment of 2 radicular cyst and dental extraction in that patient under hemostatic control by the infusion of HLA-matched platelet concentrates (PCs), which were prepared by a newly introduced cell separator named SPECTRA (COBS). The PCs prepared by SPECTRA had a great advantage in terms of reduction of contaminated white blood cell count, as compared with those prepared by other cell separators. Infusion of the SPECTRA-PCs to the patient showed dramatic effect on the shortening of bleeding time, and no excess bleeding was observed during operation. Despite these clinical improvement, ristocetin-induced platelet aggregation (RIPA) defective in the patient remained almost unchanged. To explore this conflicting result, studies on, RIPA were performed in vitro by mixing platelet and plasmas from the patient or normal individual. From this experiment, it became clear that defective RIPA found in the patient even after infusion of normal PC is mostly attributable to the presence of dysfunctional giant platelet, and not to the presence of plasma factor including inhibitor to platelet membrance components.