改良B-NHL-BFM-90方案治疗儿童青少年间变T细胞淋巴瘤的疗效分析

Efficacy of modified B-NHL-BFM-90 protocol on anaplastic T-cell lymphoma in children and adolescents

背景与目的:儿童青少年间变大细胞淋巴瘤侵袭性较强,最佳的治疗策略和方案仍需要探讨。本研究总结采用改良B-NHL-BFM-90方案治疗的儿童青少年间变T细胞淋巴瘤疗效和生存率,探讨该治疗方案的临床推广价值。方法:从2002年10月至2008年1月,18例16岁以下经病理确诊的初治间变T细胞淋巴瘤患者入组,采用改良B-NHL-BFM-90方案治疗,药物包括cyclophosphamide、vincristine、ifosfamide、etoposide、adriamycin、HD-methotrexate、vindesine、dexamethasone、cytarabine或HDcytarabine,同时每个疗程鞘内注射一次。结果:15例(83.3%)完全缓解(complete remission,CR),3例(16.7%)部分缓解(partial remission,PR),总有效率100%。中位随访31个月(4～68个月),全组3年无事件生存率(event-free survival,EFS)(87.4±8.4)%;Ⅰ/Ⅱ期患者为100%,Ⅲ/Ⅳ期患者为(85.1±9.7)%;低危组100%,中危组(88.9±10.5)%,高危组(80.0±17.9)%。治疗期间大部分患者发生Ⅲ/Ⅳ度骨髓抑制,积极对症支持治疗后均恢复。1例Ⅳ期患者CR后行自体造血干细胞移植生存至今。2例患者分别于停止治疗3个月和5个月后复发死亡。结论:改良B-NHL-BFM-90方案是治疗儿童青少年间变T细胞淋巴瘤的有效方案,其毒性患者可耐受,但需要在有经验的肿瘤中心和血液科中应用。

Background and Objective： Anaplastic T-cell lymphoma in children and adolescents is an aggressive malignant non-Hodgkin＇s lymphoma （NHL）. The optimal treatment regimen needs to be investigated. This study was to evaluate the efficacy of modified B-NHL-BFM-90 protocol on anaplastic T-cell lymphoma in children and adolescents. Methods. From October 2002 to January 2008, 18 untreated anaplastic T-cell lymphoma patients aged less than 16 years were enrolled, and treated with modified B-NHL-BFM-90 protocol including cyclophosphamide, vincristine, ifosfamide, etoposide, adriamycin, HD-methotrexate, vindesine, dexamethasone, cytarabine/HD-cytarabine. Intrathecal injection was given every course. Results. Of the 18 patients, 15 （83.3%） achieved complete remission （CR）, and three （16.7%） achieved partial remission （PR）. The patients were followed up for 4-68 months （median, 31 months）. The 3-year event- free survival （EFS） rates were （87.4±8.4）% for all patients, 100% for stage II patients, and （85.1 ±9.7）% for stage Ⅲ/Ⅳ patients; 100% for low risk group, （88.9±10.5）% for moderate risk group, and （80.0±17.9）% for high risk group. Most patients suffered from grade 3-4 myelosuppression and recovered after active support care. One patient with stage IV disease received autologous peripheral blood stem cell transplantation （PBSCT） after CR and was still alive. Two patients had tumor relapsed and died at three and five months after off treatment, respectively. Conclusions： Modified B-NHL-BFM-90 protocol, with tolerable toxicity, is an effective treatment regimen for anaplastic T-cell lymphoma in children and adolescents. It should be used in experienced cancer centers and hematological units.