Sonoda T, Solomon A, Krauss S, Cruz P, Jones F S, Levin J
Blood. 1976 Jun;47(6):983-9.
The course and treatment of a life-threatening hemorrhagic episode in a patient with hemophilia A whose plasma contained a high concentration of an inhibitor of factor VIII activity is presented. The inhibitor of factor VIII was localized to the most anodal fractions of immunoglobulin G on electrophoresis, and was thus presumed to be an antibody directed against factor VIII. No therapeutic benefit occurred with infusions of massive amounts of fresh blood and factor VIII concentrates, or with a brief course of immunosuppressive therapy. Administration of standard and activated prothrombin complex concentrates resulted in reduction of the partial thromboplastin time to almost normal values and control of hemorrhage. Eight months later, another hemorrhagic episode occurred. Although a higher titer of inhibitor of factor VIII activity was still present in the patient's plasma, a beneficial therapeutic response was again achieved with standard prothrombin complex infusions.
本文介绍了一名甲型血友病患者发生危及生命的出血发作的病程及治疗情况，该患者血浆中含有高浓度的凝血因子VIII活性抑制剂。凝血因子VIII抑制剂在电泳时定位于免疫球蛋白G的最阳极组分，因此推测其为针对凝血因子VIII的抗体。大量输注新鲜血液和凝血因子VIII浓缩物，或进行短期免疫抑制治疗均未产生治疗效果。给予标准和活化的凝血酶原复合物浓缩物后，部分凝血活酶时间降至几乎正常水平，出血得到控制。八个月后，再次发生出血发作。尽管患者血浆中仍存在较高滴度的凝血因子VIII活性抑制剂，但标准凝血酶原复合物输注再次取得了有益的治疗效果。
