中国儿童淋巴瘤协作组成熟B细胞淋巴瘤2017方案治疗儿童伯基特淋巴瘤中期疗效评价  

Mid-term efficacy of China Net Childhood Lymphoma-mature B-cell lymphoma 2017 regimen in the treatment of pediatric Burkitt lymphoma

作　　者：张梦[1] 吴攀[2] 段彦龙[1] 金玲[1] 杨菁[1] 黄爽[1] 刘英 胡波 翟晓文 王宏胜 富洋 李府[5] 杨晓梅[5] 刘安生[6] 秦爽[6] 袁晓军[7] 董玉双 刘炜 周建文 张乐萍[9] 贾月萍[9] 汪俭 屈丽君 戴云鹏[11] 管国涛[11] 孙立荣[12] 姜健[12] 刘嵘[13] 金润铭[14] 王柱军 王西阁[15] 张宝玺[16] 陈开澜[17] 庄树铨 张静 周春菊[20] 高子芬[21] 郑敏翠[2] 张永红[1] Zhang Meng;Wu Pan;Duan Yanlong;Jin Ling;Yang Jing;Huang Shuang;Liu Ying;Hu Bo;Zhai Xiaowen;Wang Hongsheng;Fu Yang;Li Fu;Yang Xiaomei;Liu Ansheng;Qin Shuang;Yuan Xiaojun;Dong Yushuang;Liu Wei;Zhou Jianwen;Zhang Leping;Jia Yueping;Wang Jian;Qu Lijun;Dai Yunpeng;Guan Guotao;Sun Lirong;Jiang Jian;Liu Rong;Jin Runming;Wang Zhujun;Wang Xige;Zhang Baoxi;Chen Kailan;Zhuang Shuquan;Zhang Jing;Zhou Chunju;Gao Zifen;Zheng Mincui;Zhang Yonghong(Medical Oncology Department,Pediatric Oncology Center,Beijing Children′s Hospital,Capital Medical University,National Center for Children′s Health,Beijing Key Laboratory of Pediatric Hematology Oncology,Key Laboratory of Major Diseases in Children,Ministry of Education,Beijing 100045,China;Department of Hematology,Hunan Children′s Hospital,Changsha 410007,China;Department of Pediatric Lymphoma,Beijing GoBroad Boren Hospital,Beijing 100070,China;Department of Hematology,Children′s Hospital of Fudan University,National Children′s Medical Center,Shanghai 201102,China;Hematology&Oncology Department,Children′s Hospital Affiliated to Shandong University,Jinan 250022,China;Department of Hematology&Oncology,Xi′an Children′s Hospital,Xi′an 710002,China;Department of Pediatric Hematology/Oncology,Xinhua Hospital,Shanghai Jiao Tong University School of Medicine,Shanghai 200092,China;Department of Hematology&Oncology,Zhengzhou Children′s Hospital,Zhengzhou 450018,China;Department of Pediatrics,Peking University People′s Hospital,Beijing 100044,China;Department of Hematology&Oncology,Anhui Children′s Hospital,Hefei 230022,China;Department of Pediatric Hematology&Endocrinology,Shandong Provincial Hospital Affiliated to Shandong First Medical University,Jinan 250021,China;Department of Pediatric Hematology&Oncology,the Affiliated Hospital of Qingdao University,Qingdao 266003,China;Department of Hematology,Children′s Hospital,Capital Pediatric Research Institute,Beijing 100020,China;Department of Pediatrics,Union Hospital,Tongji Medical College,Huazhong University of Science

机构地区：[1]国家儿童医学中心,首都医科大学附属北京儿童医院儿童肿瘤中心肿瘤内科,儿童血液病与肿瘤分子分型北京市重点实验室,儿科重大疾病研究教育部重点实验室,北京100045 [2]湖南省儿童医院血液内科,长沙410007 [3]北京高博博仁医院儿童淋巴瘤科,北京100070 [4]国家儿童医学中心复旦大学附属儿科医院血液科,上海201102 [5]山东大学附属儿童医院(济南市儿童医院)血液肿瘤科,济南250022 [6]西安市儿童医院血液肿瘤科,西安710002 [7]上海交通大学医学院附属新华医院小儿血液肿瘤科,上海200092 [8]郑州儿童医院血液肿瘤科,郑州450018 [9]北京大学人民医院儿科,北京100044 [10]安徽省儿童医院血液肿瘤科,合肥230022 [11]山东第医科大学附属山东省立医院小儿血液内分泌科,济南250021 [12]青岛大学附属医院儿童医学中心儿童血液肿瘤科,青岛26003 [13]首都儿科研究所附属儿童医院血液内科,北京100020 [14]华中科技大学同济医学院附属协和医院儿童血液与肿瘤科,武汉430022 [15]郑州大学第三附属医院血液/肿瘤科,郑州450052 [16]河北医科大学第医院儿科,石家庄050004 [17]华中科技大学同济医学院附属武汉儿童医院血液肿瘤科,武汉430016 [18]福建医科大学附属泉州第一医院儿科,泉州362002 [19]乌鲁木齐第一人民医院血液肿瘤科,乌鲁木齐830002 [20]国家儿童医学中心,首都医科大学附属北京儿童医院病理科,北京100045 [21]北京大学第三医院病理科,北京100191

出　　处：《中华儿科杂志》2022年第10期1011-1018,共8页Chinese Journal of Pediatrics

基　　金：北京市医院管理中心儿科学科协同发展中心专项(XTZD20180204)。

摘　　要：目的分析儿童伯基特淋巴瘤(BL)的临床特点, 总结中国儿童淋巴瘤协作组成熟B细胞淋巴瘤2017方案(CNCL-B-NHL-2017)的中期疗效。方法回顾性收集中国儿童淋巴瘤协作组2017年5月至2021年4月收治的436例年龄≤18岁BL患儿临床资料, 均按照CNCL-B-NHL-2017方案分层治疗。分析患儿发病时临床特点, 比较不同临床分期、危险度分组患儿的治疗效果, 采用Kaplan-Meier方法进行生存分析, Cox回归分析影响预后的危险因素。结果 436例患儿发病年龄6.0(4.0, 9.0)岁, 男368例(84.4%)、女68例(15.6%)。临床分期Ⅰ、Ⅱ、Ⅲ、Ⅳ期分别为4例(0.9%)、30例(6.9%)、217例(49.8%)、185例(42.4%), 危险度A、B1、B2、C1、C2组分别为1例(0.2%)、46例(10.6%)、19例(4.4%)、285例(65.4%)、85例(19.5%)。全组患儿中63例(14.4%)单纯化疗, 373例(85.6%)在化疗基础上联合应用利妥昔单抗。21例(4.8%)治疗中进展, 3例(0.7%)复发, 13例(3.0%)治疗相关死亡。随访时间24.0(13.0, 35.0)个月, 全组患儿2年无事件生存率(EFS)为(90.9±1.4)%, A、B1、B2、C1、C2组2年EFS分别为100.0%、100.0%、(94.7±5.1)%、(90.7±1.7)%、(85.9±4.0)%, A、B1、B2组整体高于C1组(χ^(2)=4.16, P=0.041)和C2组(χ^(2)=7.21, P=0.007)。单纯化疗组和利妥昔单抗联合化疗组2年EFS分别为(79.3±5.1)%、(92.9±1.4)%, 差异有统计学意义(χ^(2)=14.23, P<0.001)。临床分期Ⅳ期(包括白血病期)、血清乳酸脱氢酶(LDH)>正常值4倍、中期评估有瘤灶为预后不良的危险因素(HR=1.38、1.23、8.52, 95%CI 1.05~1.82、1.05~1.43、3.96~18.30)。结论 CNCL-B-NHL-2017方案对儿童BL疗效显著, 联合应用利妥昔单抗可以使疗效进一步提高。Objective To analyze the clinical characteristics of children with Burkitt lymphoma(BL)and to summarize the mid-term efficacy of China Net Childhood Lymphoma-mature B-cell lymphoma 2017(CNCL-B-NHL-2017)regimen.Methods Clinical features of 436 BL patients who were≤18 years old and treated with the CNCL-B-NHL-2017 regimen from May 2017 to April 2021 were analyzed retrospectively.Clinical characteristics of patients at disease onset were analyzed and the therapeutic effects of patients with different clinical stages and risk groups were compared.Survival analysis was performed by Kaplan-Meier method,and Cox regression was used to identify the prognostic factors.Results Among 436 patients,there were 368(84.4%)males and 68(15.6%)females,the age of disease onset was 6.0(4.0,9.0)years old.According to the St.Jude staging system,there were 4 patients(0.9%)with stageⅠ,30 patients(6.9%)with stageⅡ,217 patients(49.8%)with stageⅢ,and 185 patients(42.4%)with stageⅣ.All patients were stratified into following risk groups:group A(n=1,0.2%),group B1(n=46,10.6%),group B2(n=19,4.4%),group C1(n=285,65.4%),group C2(n=85,19.5%).Sixty-three patients(14.4%)were treated with chemotherapy only and 373 patients(85.6%)were treated with chemotherapy combined with rituximab.Twenty-one patients(4.8%)suffered from progressive disease,3 patients(0.7%)relapsed,and 13 patients(3.0%)died of treatment-related complications.The follow-up time of all patients was 24.0(13.0,35.0)months,the 2-year event free survival(EFS)rate of all patients was(90.9±1.4)%.The 2-year EFS rates of group A,B1,B2,C1 and C2 were 100.0%,100.0%,(94.7±5.1)%,(90.7±1.7)%and(85.9±4.0)%,respectively.The 2-year EFS rates was higher in group A,B1,and B2 than those in group C1(χ^(2)=4.16,P=0.041)and group C2(χ^(2)=7.21,P=0.007).The 2-year EFS rates of the patients treated with chemotherapy alone and those treated with chemotherapy combined with rituximab were(79.3±5.1)%and(92.9±1.4)%(χ^(2)=14.23,P<0.001)respectively.Multivariate analysis showed that stageⅣ(inclu

关 键 词：伯基特淋巴瘤 儿童 预后 

分 类 号：R733.1[医药卫生—肿瘤]