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目的:难治性获得性血友病A(AHA)对激素与常用的免疫抑制剂无反应,出血危险性大,我们试用雷帕霉素治疗1例难治性AHA患者。方法:用全自动血凝仪测定凝血常规以及相关凝血因子,Bethesda试验检测因子Ⅷ抑制物。结果:该患者的凝血因子Ⅷ活性仅为1%,因子Ⅷ抑制物效价达87.5Bethesda单位。该患者对激素与硫唑嘌呤治疗无反应,2次标准剂量的美罗华治疗仅暂时有效,表现为顽固性AHA;改用雷帕霉素(2mg/d,3个月后减为1mg/d,疗程6个月)后出血完全停止,凝血指标亦恢复正常,但停药后复发。重新给予雷帕霉素后再次缓解,逐渐减量后停药,随访1年中凝血常规检查维持在正常范围。结论:雷帕霉素是一种安全、有效的新型免疫抑制剂,本文首次报道1例顽固性AHA患者在对激素与常用的免疫抑制剂无效时改用雷帕霉素后治愈。
OBJECTIVE: Refractory acquired hemophilia A (AHA) is refractory to hormones and commonly used immunosuppressive agents and has a high risk of bleeding. We tested rapamycin in 1 refractory AHA patient. Methods: Coagulation routine and related coagulation factors were determined by automatic coagulation analyzer, and factor Ⅷ inhibitor was detected by Bethesda test. RESULTS: The patient had only 1% coagulation factor VIII activity and the factor VIII inhibitor titer was 87.5 Bethesda units. The patient did not respond to the treatment of azathioprine with hormones. The 2 standard doses of rituximab were only temporarily effective and showed refractory AHA; instead, rapamycin (2 mg / d, 3 mg / d, Course of treatment for 6 months) completely stopped bleeding, coagulation index also returned to normal, but relapse after stopping. After re-given rapamycin again relieved gradually tapering after withdrawal, follow-up 1 year coagulation routine examination remained in the normal range. Conclusion: Rapamycin is a safe and effective new immunosuppressant. In this paper, we report for the first time that one patient with intractable AHA is cured after switching to rapamycin when the hormone and commonly used immunosuppressive drugs are ineffective.