环磷酰胺序贯治疗伴胸腺瘤的重症肌无力50例临床分析

Cyclophosphamide therapy for myasthenia gravis with thymoma： a clinical analysis of 50 cases

目的 观察环磷酰胺治疗伴胸腺瘤的重症肌无力的疗效和不良反应.方法 对胸腺切除术后病理证实为胸腺瘤的重症肌无力患者50例进行环磷酰胺序贯治疗.先以环磷酰胺0.20 g/d（2次/周）静脉滴注,逐渐增加剂量至0.80 g/次（1次/周）,至总量10 g时继续0.80 g/次（1次/月）,直至治疗总剂量20.4 g.根据临床绝对评分和相对评分判定肌无力严重程度及评价疗效,同时观察药物不良反应.结果 与治疗前临床绝对评分[（20.44±8.80）分]相比,治疗总剂量达到6 g、10 g、20.4 g时的临床绝对评分分别为（14.20±8.68）分、（10.96±8.27）分、（3.84±4.44）分,明显降低（P〈0.05）.总剂量达6 g、10 g时的临床绝对评分与20.4 g时相比,差异有统计学意义（P〈0.05）.50例患者在环磷酰胺总量达到6 g、10 g和20.4 g时有效率分别为70％、78％和94％,随剂量增加疗效越明显,不良反应轻微且可耐受.结论 环磷酰胺治疗伴胸腺瘤的重症肌无力疗效明显,总剂量越大,疗效越好;环磷酰胺总剂量达20.4 g时效果显著、安全,值得推荐使用.

Objective To observe the curative effects of cyclophosphamide treatment in myasthenia gravis （MG） with thymoma and adverse reactions in the treatment. Methods A total of 50 eases of myasthenia gravis patients that had proved thymoma by pathology after thymectomy were given cyclophosphamide sequential therapy. Cyclophospbamide was given intravenously at a dose of 0.20 g/d （twice a week）, gradually increased to 0.80 g/d （once a week） till the total dose was 10 g, and continued at a dose of 0.80 g/d （once a month） until the total dose was 20.4 g. The severity and efficacy of myasthenia were evaluated according to clinical absolute score and relative score, and adverse drug reactions were observed. Results Compared with the clinical absolute scores of prior treatment （20.44 ± 8.80）, when the total treatment dose reaches 6 g, 10 g, 20.4 g, the clinical absolute scores were （14.20±8.68）, （10.96 ± 8.27） and （3.84 ± 4.44） respectively, which were significantly reduced （P 〈 0.05）. Comparing the clinical absolute scores of the total dose 6 g and 10 g with 20.4 g, the difference was statistically significant （P 〈 0.05）. The effective rate of 50 cases at the total dose 6 g, 10 g and 20.4 g were 70%, 78% and 94% respectively. With the increase of the cyclophosphamide dosages, the curative effect was more obvious, and the adverse reactions were mild and tolerable. Conclusions The curative effect of cyclophosphamide medicine for MG with thymoma is obviously. The greater the total dose, the better the curative effect. When cyclophosphamide total dose reaches 20.4 g, the curative effect is remarkable with less adverse reactions. It was worthy of recommendation.