短疗程、高强度化疗方案治疗儿童青少年B细胞非霍奇金淋巴瘤的疗效

Efficacy of short-term and intensive chemotherapy for the treatment of childhood and adolescent B cell non-Hodgkin’s lymphoma

目的评价德国NHL-BFM-90方案治疗中国儿童青少年B细胞非霍奇金淋巴瘤(B-NHL)的疗效和毒性.方法20岁以下的儿童青少年B-NHL患者42例,其中伯基特淋巴瘤18例,弥漫性大B细胞淋巴瘤16例,间变性大细胞淋巴瘤8例;早期病例(Ⅱ期)10例,晚期病例(Ⅲ,Ⅳ期)32例.所有患者均接受NHL-BFM-90方案化疗.根据临床分期、血清乳酸脱氢酶(LDH)的水平等预后因素,分为低、中和高危3组.低危组(Ⅱ期患者)接受A和B疗程交替化疗4个疗程.中危组(Ⅲ期和LDH＜500U/L的Ⅳ期患者)接受AA和BB疗程交替化疗6个疗程.高危组[LDH＞500 U/L的Ⅳ期患者和中危组两疗程AA、BB治疗后不能完全缓解(CR)的患者]接受AA、BB和CC疗程交替化疗6个疗程.每个疗程间隔18～21 d.结果37例(88%)患者获得CR,5例(12%)患者获得部分缓解(PR),其中1例接受自体造血干细胞移植,3例行残留病灶放疔,1例观察.主要不良反应为骨髓抑制和黏膜炎,以AA、BB和CC疗程明显,但均可耐受.中位随访20(4～89)个月,按Kaplan-Meier方法进行生存率统计,2年全组无事件生存(EFS)率为86.24%,早期患者为100%,晚期患者为80.95%.结论短疗程、高强度化疗结合中枢神经系统预防的治疗策略能明显改善儿童青少年B-NHL患者的疗效和生存率,特别是对晚期患者的疗效更明显.

Objectives To evaluate the efficacy and toxicity of the B-NHL-BFM-90 protocol in the treatment of Chinese childhood and adolescent B-cell non-Hodgkin＇s lymphomas（B-NHL）. Methods Forty-two untreated childhood and adolescent B-NHL were enrolled in the present study. Of them 18 cases were Burkitt＇s lymphoma, 16 diffuse large B cell lymphoma and 8 anaplastic lymphoma. There were 10 cases in stage Ⅱ and 32 in stage Ⅲ /Ⅳ. The patients were grouped by risk factors into low, medium and high risk groups. All patients were treated with the B-NHL-BFM 90 （ Berlin-Frankfurt- Miinster） protocol. The low risk group received A, B courses for 4 cycles, the medium risk group AA, BB courses for 6 cycles, and the high risk group AA,BB, CC courses for 6 cycles. Results Complete remission （CR） was obtained in 37 patients （ 88% ） , and partial remission （PR） in 5 （ 12% ）. Of the 5 PR patients, 1 received autologous hematopoietic stem cell transplantation, 3 received radiotherapy for residual disease and 1 just under watching. Major toxicity was myelosuppression and mucositis, especially in AA, BB and CC cycles, but was tolerant and manageable. Median follow-up was 20 （4 - 89） months. Kaplan-Meier method was used to analyse survival data. Two vear event free survival （EFS） for all patients was 86.24% , being 100% for stage Ⅱ and 80.95% for stage Ⅲ/Ⅳ. Conclusion Short term and intensive chemotherapy can improves the efficacy and survival rate of childhood and adolescent B-NHL, especially for advanced stage patients.