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46例儿童弥漫性大B细胞淋巴瘤的临床特征及治疗结果

[Clinical characteristics of 46 pediatric diffuse large B-cell lymphoma and treatment outcome].

作者信息

Huang S, Yang J, Jin L, Duan Y L, Zhang M, Zhang N N, Li Q, Zhang N, Zhou C J, Zhang Y H

机构信息

Department of Hematology and Oncology, Beijing Children's Hospital, Capital Medical University, Beijing 100045, China.

Department of Pathology, Beijing Children's Hospital, Capital Medical University, Beijing 100045, China.

出版信息

Zhonghua Er Ke Za Zhi. 2019 Oct 2;57(10):774-779. doi: 10.3760/cma.j.issn.0578-1310.2019.10.009.

Abstract

To summarize the clinical data of diffuse large B-cell lymphoma (DLBCL) in children and to evaluate the efficacy of Beijing Children's Hospital B cell lymphoma protocol in the treatment of pediatric DLBCL. The data (clinical, pathology, lab and image data) of 46 pediatric DLBCL admitted to the treatment group of Beijing Children's Hospital from January 2005 to June 2017 were collected and analyzed retrospectively. According to the risk factors of staging, existence of poor prognosis genes and giant tumors, stratified treatment was carried out according to the international standard modified LMB89 regimen with high dose and short course. The Kaplan-Meier method was used to calculate the event free survival (EFS) and the overall survival (OS). (1) Among the 46 cases, there were 33 males and 13 females. The median age was 8.0 years. The time from the initial symptom onset to the diagnosis was more than 15 days in 45 children. Fourteen cases had B group symptoms (fever, night sweat, and weight lost), 25 cases had extranodal disease, 39 cases were stage Ⅲ and Ⅳ, 12 cases had bone marrow involvement, 3 cases had jawbone involvement. Thirty cases were group B and 16 cases were group C in the treatment group. (2) Initial symptoms: 6 cases had cervical mass, 20 cases had abdominal mass, 10 had abdominal pain with acute abdomen, 8 cases had fever, 2 cases had snore or upper respiratory tract obstruction. (3) Pathology result: 40 cases were germinal center B cell DLBCL, 6 cases were non germinal center B cell DLBCL, no case had the MYC gene rupture, double hit lymphoma and triple hit lymphoma. (4) Complication and evaluation: the tumor lysis syndrome was seen in 3 cases initially, severe infection and delayed treatment was seen in 1 case, no treatment related death. The first evaluation showed all cases were sensitive to chemotherapy (shrink>25%), the second evaluation showed 1 case had residual disease, the others were complete remission. (5) Treatment and outcome: the 5 year-EFS was the same with 5 year-OS, both were (97.8±2.2) %. Two cases relapsed after treatment off, early relapse was seen in 1 case, and died because of abandoning treatment. Late relapse was seen in 1 case and got a complete remission after Rituximab+group C protocol treatment. Pediatric DLBCL was common in school aged boys, most cases were at middle and late stage at the time of diagnosis. DLBCL had a good prognosis after the treatment with Beijing Children's Hospital's B cell lymphoma protocol, but late relapse could be seen.

摘要

总结儿童弥漫性大B细胞淋巴瘤(DLBCL)的临床资料,评估北京儿童医院B细胞淋巴瘤方案治疗儿童DLBCL的疗效。回顾性收集并分析2005年1月至2017年6月在北京儿童医院治疗组住院的46例儿童DLBCL的资料(临床、病理、实验室及影像学资料)。根据分期、预后不良基因存在情况及巨大肿瘤等危险因素,参照国际标准改良LMB89方案进行大剂量短疗程分层治疗。采用Kaplan-Meier法计算无事件生存(EFS)和总生存(OS)。(1)46例中,男33例,女13例。中位年龄8.0岁。45例患儿从出现初始症状到确诊时间超过15天。14例有B组症状(发热、盗汗、体重减轻),25例有结外病变,39例为Ⅲ、Ⅳ期,12例有骨髓受累,3例有颌骨受累。治疗组中30例为B组,16例为C组。(2)初始症状:6例有颈部肿块,20例有腹部肿块,10例有腹痛伴急腹症,8例有发热,2例有打鼾或上呼吸道梗阻。(3)病理结果:40例为生发中心B细胞DLBCL,6例为非生发中心B细胞DLBCL,无MYC基因断裂、双打击淋巴瘤及三打击淋巴瘤病例。(4)并发症及评估:3例初诊时出现肿瘤溶解综合征,1例出现严重感染及延迟治疗,无治疗相关死亡。首次评估显示所有病例对化疗敏感(缩小>25%),第二次评估显示1例有残留病灶,其余均为完全缓解。(5)治疗及转归:5年EFS与5年OS相同,均为(97.8±2.2)%。2例治疗停药后复发,1例为早期复发,因放弃治疗死亡。1例为晚期复发,经利妥昔单抗+C组方案治疗后完全缓解。儿童DLBCL在学龄期男孩中常见,多数病例确诊时处于中晚期。采用北京儿童医院B细胞淋巴瘤方案治疗后DLBCL预后良好,但可见晚期复发。

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