[改良B-NHL-BFM-90方案对中国儿童及青少年伯基特淋巴瘤的疗效]

[Efficacy of modified B-NHL-BFM-90 protocol on Burkitt's lymphoma in Chinese children and adolescents].

作者信息

Sun Xiao-Fei, Zhen Zi-Jun, Liu Dong-Geng, Xia Yi, Xiang Xiao-Juan, Chen Xiao-Qin, Ling Jia-Yu, Zheng Lei, Luo Wen-Biao, Lin Hui, He You-Jian, Guan Zhong-Zhen

机构信息

State Key Laboratory of Oncology in South China, Guangzhou, Guangdong, 510060, PR China.

出版信息

Ai Zheng. 2007 Dec;26(12):1339-43.

PMID:18076797

Abstract

BACKGROUND & OBJECTIVE: Burkitt's lymphoma is an aggressive non-Hodgkin's lymphoma (NHL) and often involves bone marrow and central nerve system. The efficacy of CHOP regimen on Burkitt's lymphoma is poor. The optimal chemotherapy regimen needs to be investigated. This study was to evaluate the efficacy of modified B-NHL-BFM-90 protocol on Burkitt's lymphoma in children and adolescents, and observe the survival status.

METHODS

From Oct. 1999 to Nov. 2006, 31 untreated Burkitt's lymphoma patients aged less than 20 were enrolled. The median age of these patients was 5 (range, 1.5-20 years old). Of the 31 patients, 20 (64.5%) were male, 11 (35.5%) were female. According to St Jude staging system, 1 (3.2%) was at stage I, 6 (19.4%) at stage II, 8 (25.8%) at stage III, 16 (51.6%) at stage IV; 24 (77.4%) were at stage III/IV. According to clinical stage, lactate dehydrogenase (LDH) level and treatment response, these patients were divided into low, moderate and high risk groups. They received modified B-NHL-BFM-90 protocol: cytotoxic drugs such as cyclophosphamide, vincristine, ifosfamide, etoposide, adriamycin, HD-methotrexate, vindesin, dexamethasone, cytarabinec/HD-cytarabine and intrathecal injection.

RESULTS

One patient died of tumor lysis syndrome during prophase. The efficacy was evaluable in 30 patients. Of the 30 patients, 25 (83.3%) achieved complete remission (CR), 3 (10.0%) achieved partial remission (PR), 2 (6.7%) had progressive disease (PD)û 1 had tumor relapse. Grade 3-4 myelosuppression occurred in most patients and were recovered by active support care and did not affect next course of chemotherapy. At a median follow-up of 33 months (range, 3-98 months), the 3-year event-free survival (EFS) rate was 86.0% for all patients, with 100% for stage I/II patients and 82.1% for stage III/IV patients, 100% for low risk group, 92.0% for moderate risk group, and 70.0% for high risk group.

CONCLUSIONS

Modified B-NHL-BFM-90 protocol can improve the responses and survival of Burkitt's lymphoma in Chinese children and adolescents, with tolerable toxicity. It should be used in the experienced cancer center and hematological unit.

摘要

背景与目的

伯基特淋巴瘤是一种侵袭性非霍奇金淋巴瘤（NHL），常累及骨髓和中枢神经系统。CHOP方案治疗伯基特淋巴瘤疗效欠佳，需探寻最佳化疗方案。本研究旨在评估改良B-NHL-BFM-90方案治疗儿童及青少年伯基特淋巴瘤的疗效，并观察生存状况。

方法

1999年10月至2006年11月，纳入31例年龄小于20岁的初治伯基特淋巴瘤患者。这些患者的中位年龄为5岁（范围1.5 - 20岁）。31例患者中，男性20例（64.5%），女性11例（35.5%）。根据圣裘德分期系统，I期1例（3.2%），II期6例（19.4%），III期8例（25.8%），IV期16例（51.6%）；III/IV期24例（77.4%）。根据临床分期、乳酸脱氢酶（LDH）水平及治疗反应，将这些患者分为低、中、高危组。他们接受改良B-NHL-BFM-90方案：使用环磷酰胺、长春新碱、异环磷酰胺、依托泊苷、阿霉素、大剂量甲氨蝶呤、长春地辛、地塞米松、阿糖胞苷/大剂量阿糖胞苷等细胞毒性药物及鞘内注射。

结果

1例患者在前期死于肿瘤溶解综合征。30例患者疗效可评估。30例患者中，25例（83.3%）达到完全缓解（CR），3例（10.0%）达到部分缓解（PR），2例（6.7%）疾病进展（PD），1例肿瘤复发。多数患者发生3 - 4级骨髓抑制，经积极支持治疗后恢复，未影响下一疗程化疗。中位随访33个月（范围3 - 98个月），所有患者3年无事件生存率（EFS）为86.0%，I/II期患者为100%，III/IV期患者为82.1%，低危组为100%，中危组为92.0%，高危组为70.0%。