加熱第Ⅷ因子濃縮製剤，Haemate Pの止血管理下に抜歯を行ったType ⅡB von Willebrand病の1例

Abstract

A dental surgery case is described of a 12-year-old boy with type ⅡB von Willebrand's disease (vWD), suffering from toothache caused by follicular cyst with infection in the mandible. Prior to the surgery, a hemostatic effect of Haemate P was studied. After the infusion of Haemate P (84 U/kg body weight of ristocetin cofactor (Rcof.) ; 28 U/kg of factor Ⅷ activity (F. Ⅷ : C)), F. Ⅷ : C levels rose from 0.6 U/ml to 1.64 U/ml at 1 hour, 1.08 U/ml at 24 hours and 0.9 U/ml at 48 hours, respectively. The levels of Rcof. inreased from 0.24 U/ml to 2.75 U/ml at half an hour after the infusion and then gradually decreased to 0.44 U/ml at 48 hours. The levels of botrocetin cofactor (Bcof.) increased from 0.4 U/ml to 2.6 U/ml at half an hour after the infusion and then gradually decreased to the preinfusion level at 48 hours. Before the infusion, the large multimers were absent in the plasma, whereas all multimers were present in the platelets. After the infusion, the large multimers in the plasma appeared for several hours and gradually disappeared at 48 hours. Surgical procedures - an extirpation of the cyst in the mandibular bone and the extractions of 3 deciduous teeth (both sides of the second mandibular lower deciduous molars and the right upper second deciduous molar) and 1 supernumerary tooth in the maxilla ― were performed after the infusion of Haemate P (56 U/kg body weight of Rcof. ; 18 U/kg body weight of F. Ⅷ : C). Further infusions of Haemate P were given every 12 hours for 2 days and every 24 hours for the following 3 days. After each infusion of Haemate P, there was 2-3 fold increase of 3 activities of F. Ⅷ/vWF. During the course of Haemate P infusions, there was no significant side effect.