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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 & OBJECTIVE: The aggressiveness of large cell lymphoma in children and adolescents is still high. The best treatment strategies and protocols still need to be explored. This study summarized the efficacy and survival rate of T-cell lymphoma in children and adolescents treated with modified B-NHL-BFM-90 regimen and explored the clinical value of this regimen. Methods: From October 2002 to January 2008, 18 patients with pathologically diagnosed T-cell lymphoma under the age of 18 were enrolled in this study. The patients were treated with modified B-NHL-BFM-90 regimen. The drugs included cyclophosphamide, vincristine, ifosfamide, etoposide, adriamycin, HD-methotrexate, vindesine, dexamethasone, cytarabine, or HDcytarabine with intrathecal injections once per course of treatment. Results: Complete remission (CR) was achieved in 15 cases (83.3%) and partial remission (PR) in 3 cases (16.7%). The total effective rate was 100%. The median follow-up was 31 months (range, 4 to 68 months). The 3-year event-free survival (EFS) was 87.4 ± 8.4% in all patients and 100% in stage Ⅰ / Ⅱ. The patients were (85.1 ± 9.7)%, 100% in low risk group, 88.9 ± 10.5% in intermediate risk group and 80.0 ± 17.9% in high risk group. During the treatment of most patients with Ⅲ / Ⅳ degrees of bone marrow suppression, active symptomatic and supportive treatment were restored. One case of stage Ⅳ patients survived CR after autologous stem cell transplantation. Two patients relapsed after 3 and 5 months of discontinuation of treatment, respectively. CONCLUSIONS: The modified B-NHL-BFM-90 regimen is an effective regimen for treating anaplastic T-cell lymphoma in children and adolescents. Toxicity is tolerated in patients with T-cell lymphoma, but needs to be used in experienced oncology centers and hematology.