累积大剂量环磷酰胺治疗伴发胸腺瘤重症肌无力

Clinical observation of high cumulative doses of cyclophosphamide in the treatment of myasthenia gravis patients with thymoma

目的观察累积大剂量环磷酰胺(CTX)治疗伴胸腺瘤重症肌无力(MG)的有效性和安全性。方法对2006年5月至2018年5月本中心应用CTX治疗的伴胸腺瘤MG患者进行回顾性分析,选取输注药物总量≥30 g的患者为研究对象,记录MG临床相对评分、不良反应等。结果应用CTX达30 g后MG相对评分好转≥95%者11例,80%~95%者4例,50%~80%者2例,总有效率100%,总治愈率88.2%。治疗过程中,出现27例次药物不良反应,白细胞减少8例,丙氨酸氨基转移酶升高7例,恶心6例,疲乏1例,出汗增多1例,月经推迟1例,闭经1例,带状疱疹1例,高调耳鸣1例。结论应用CTX治疗伴发胸腺瘤MG,累积剂量达30 g时可获得显著疗效;白细胞减少、肝损害为主要不良反应,给予对症处理后可顺利完成治疗。

Objective To observe the efficacy and safety of accumulated high dose cyclophosphamide (CTX) therapy in myasthenia gravis( MG) patients with thymoma. Methods Data from MG patients with thymoma who underwent CTX pulse therapy in our center from May 2006 to May 2018 were retrospectively reviewed. Patients whose cumulative CTX dose reached to 30 g or more were selected as objects. Clinical data,including clin ical relative score and adverse effects of MG with thymoma,were recorded. Results The clinical relative scores declined more than 95% in 11 cases,80%~95% in 4 cases,50%~80% in 2 cases. The effective ratio was 100%,and the curative ratio was 88.2%. Twenty seven adverse events were documented. These side effects were leukopenia(n = 8), elevated alanine transaminase levels(n = 7), nausea(n = 6), fatigue(n = 1), increased sweating(n = 1), delayed menstruation(n = 1), amenorrhea(n = 1), herpes zoster(n = 1), and high pitch tinnitus(n = 1). Conclusions Intravenous pulse CTX is significantly effective in the treatment of MG patients with thymoma,w hen the total cumulative doses reached to 30 g. Leukopenia and liver damage were the common adverse reactions. When adverse events were managed symptomatically,the treatment could be completed successfully.