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Title: [Clinical characteristics of 46 pediatric diffuse large B-cell lymphoma and treatment outcome].
Author: Huang S, Yang J, Jin L, Duan YL, Zhang M, Zhang NN, Li Q, Zhang N, Zhou CJ, Zhang YH.
Journal: Zhonghua Er Ke Za Zhi; 2019 Oct 02; 57(10):774-779. PubMed ID: 31594064.
Abstract:
Objective: 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. Methods: 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). Results: (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. Conclusions: 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. 目的： 总结儿童弥漫大B细胞淋巴瘤（DLBCL）的临床及病理资料，探讨北京儿童医院B细胞淋巴瘤方案对DLBCL的疗效。 方法： 回顾性收集2005年1月至2017年6月进入北京儿童医院治疗组的46例DLBCL患儿的资料（临床、病理、实验室、影像学），依据患儿的分期、是否存在预后不良基因及巨大瘤灶等危险因素，按照改良的LMB89方案进行分层治疗，应用Kaplan-Meier方法计算无事件生存率（EFS）及总生存率（OS）。 结果： （1）46例患儿中男33例、女13例，初诊时中位年龄8.0岁；45例患儿从发病至确诊时间≥15 d；14例存在B组症状（即发热、盗汗、体重下降）；25例存在结外侵犯；Ⅲ期、Ⅳ期患儿共39例；12例存在骨髓侵犯，3例颌面部侵犯；治疗分组B组患儿30例、C组16例。（2）首发症状：6例颈部包块，20例腹部包块，10例腹痛伴急腹症，8例发热，2例存在打鼾或上呼吸道梗阻。（3）病理：生发中心来源40例，非生发中心来源6例；46例患儿均未见MYC断裂，无双打击及三打击淋巴瘤。（4）并发症及评估：3例合并肿瘤溶解综合征，1例存在重症感染而延迟化疗；无治疗相关死亡。早期评估全部患儿缩小均>25%，中期评估有残留病灶者1例，停药前检查46例患儿全部完全缓解。（5）疗效及预后：5年EFS与5年OS相同，均为（97.8±2.2）%，停药后复发2例，1例为早期复发患儿，复发后放弃治疗死亡；1例为晚期复发患儿，复发后再次给予利妥昔单克隆抗体+治疗组C组方案化疗后再次达完全缓解。 结论： DLBCL在学龄期男童多见，就诊时多为中晚期，北京儿童医院B细胞淋巴瘤方案对儿童DLBCL疗效显著，但可见晚期复发病例。.

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