血液疾患における口腔出血管理に関する研究 : 第19報 DDAVP輸注による先天性第V, VIII因子合併欠乏症の口腔出血管理

概要

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Congenital deficiencies of both factor V and VIII represent a rare blood coagulation disorder. The hemorrhagic management of this disease has been performed by transfusion of fresh plasma and factor VIII concentrate by this time.<BR>In this report, the administration of 1-deamino-8-D-arginine-vasopressin (DDAVP), which is effective for the bleeding in patients with mild and moderate hemophilia A and von Willebrands disease, resulted in the hemostasis of oral bleeding in two patients with this disorder. Factor VIII coagulability (VIII: C) and von Willebrand factor antigen (vWF: Ag) rose significantly but factor V coagulability in both cases showed no change after DDAVP administration. These findings suggested that DDAVP might have other hemostatic effects of unknown mechanism in addition to the rise of plasma VIII: C and v WF: Ag levels.