改良NHL—BFM-90方案治疗儿童及青少年伯基特淋巴瘤和弥漫大B细胞淋巴瘤的远期疗效  

Outcome of children and adolescents with Burkitt lymphoma and diffuse large B cell lymphoma treated with a modified NHL-BFM-90 protocol

作　　者：孙晓非[1] 甄子俊[1] 夏奕[2] 林素暇[3] 朱佳[1] 王娟[1] 路素英[1] 孙斐斐[1] 陈燕[1] 张飞[1] 蔡瑞卿[1] 李鹏飞[1] 

机构地区：[1]中山大学肿瘤防治中心儿童肿瘤科,华南肿瘤学重点实验室,广州510060 [2]中山大学肿瘤防治中心内科 [3]中山大学肿瘤防治中心病理科

出　　处：《中华血液学杂志》2013年第12期1032-1037,共6页Chinese Journal of Hematology

摘　　要：目的评估改良NHL—BFM-90方案治疗儿童及青少年伯基特淋巴瘤（BL）和弥漫大B细胞淋巴瘤（DLBCL）的疗效及安全性。方法病理确诊的初治BL82例和DLBCL患者56例纳入研究，根据临床分期、血清乳酸脱氢酶水平和化疗早期疗效分为低危、中危和高危组，接受改良NHL—BFM-90方案治疗。结果全部138例患者中，男105例、女33例，中位年龄7．5（1．5～20．0）岁；BL82例，DLBCL56例；Ⅲ／Ⅳ期占76．1％；低危组31例，中危组38例，高危组69例。化疗后完全缓解率为90．6％。中位随访时间50（1～158）月，共19例（13．8％）患者死亡。全组5年总生存（OS）率和无事件生存（EFS）率均为85．8％；Ⅰ／Ⅱ期患儿5年EFS率（97．1％）优于Ⅲ／Ⅳ期（82．1％），差异有统计学意义（P=0．039）；低危、中危、高危组5年EFS率差异无统计学意义（分别为96．7％、86．8％、80．2％，P=0．135）；BL、DLBCL组5年EFS率差异无统计学意义（85．2％对86．9％，P=0．635）。化疗主要不良反应为骨髓抑制，对症处理均可恢复。结论改良NHL—BFM-90方案治疗儿童及青少年BL和DLBCL疗效满意，可明显改善晚期患者的生存率。Objective To evaluate the efficacy of a modified NHL-BFM-90 protocol in childhood and adolescence with Burkitt lymphoma （BL） and diffuse large B-cell lymphoma （DLBCL）. Methods A total of 138 de novo patients with BL and DLBCL were enrolled. All patients were stratified into low （R1）, intermediate （R2） and high risk （R3） groups based on the stage, chemotherapy response and LDH level, and treated with a modified NHL-BFM 90 protocol. Results Of the 138 patients, 105 were boys and 33 girls, with a median age at diagnosis of 7.5 yr （range 1.5 to 20.0 yr）. Eighty-two cases were BL, 56 cases DLBCL. The patients with stage ]I/IV accounted for 76.1%. Thirty-one patients were assigned to group R1, 38 patients group R2, and 69 patients group R3. Complete remission （CR） after chemotherapy was 90.6%. At a median follow-up of 50 months （1-158 months）, a total of 19 patients died of disease. The 5- year event free survival （EFS） and overall survival （OS） for the entire group were 85.8%, 85.8% respectively. 5- year EFS was 97.1% for stage Ⅰ/Ⅱ, 82.1% for stage Ⅲ/Ⅳrespectively （P=0.039）; and 96.7%, 86.8% and 80.2% for groups R1, R2 and R3 respectively （P=0.135）; and 85.2% and 86.9% for BL and DLBCL respectively （P=0.635）. Major toxicity was myelosuppression, which was tolerant and manageable. Conclusions That the modified NHL-BFM-90 protocol was highly effective for children and adolescents with BL and DLBCL, and especially improved the survival of the advanced patients.

关 键 词：伯基特淋巴瘤 淋巴瘤 大B细胞 弥漫性 儿童 治疗结果 

分 类 号：R733.1[医药卫生—肿瘤]