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获得性血友病患者急性出血发作的发病率和死亡率极高。人FⅧ与PCCs分别因为交叉反应甚高及副作用大而使应用受到限制。本文回顾性分析12例获得性血友病患者的15次急性出血发作及用猪FⅧ治疗的资料,以确认猪FⅧ的治疗效果。病例来自英国的8个医疗中心。男7例,女5例,平均年龄54岁(18～73岁)。12例中结缔组织或自身免疫性疾病3例,妊娠相关疾病3例,其他6例。其中11例各有1次出血发作,1例先后4次出血。常见的出血部位为肌肉、皮内和尿道,40%患者发生多部位出血。
The incidence and mortality of acute bleeding episodes in acquired hemophilia patients are extremely high. Human F Ⅷ and PCCs are limited because of their high cross-reactivity and large side effects. This retrospective analysis of 12 cases of acquired hemophilia in patients with 15 episodes of acute bleeding and the use of FⅧ treatment of pigs to confirm the treatment of pig F VIII. The cases are from eight medical centers in the United Kingdom. There were 7 males and 5 females, with an average age of 54 years (18-73 years). In 12 cases, there were 3 cases of connective tissue or autoimmune diseases, 3 cases of pregnancy-related diseases and 6 cases of other diseases. Among them, 11 cases had one episode of bleeding and one case had bleeding 4 times successively. Common bleeding sites for muscle, intradermal and urethral, 40% of patients with multiple parts of bleeding.