儿童成熟B细胞非霍奇金淋巴瘤临床特点及预后分析  

Clinical features and prognosis of children with mature B-cell non-Hodgkin's lymphoma: an analysis of 28 cases

作　　者：陈再生[1] 郑湧智 陈以乔[1] 高琴丽[1] 李健[1] 沈建箴[2] CHEN Zai-Sheng;ZHENG Yong-Zhi;CHEN Yi-Qiao;GAO Qin-Li;LI Jian;SHEN Jian-Zhen(Fujian Institute of Hematology/Fujian Provincial Key Laboratory on Hematology/Fujian Medical University Union Hospital, Fuzhou 350001, China)

机构地区：[1]福建省血液病研究所/福建省血液病学重点实验室/福建医科大学附属协和医院小儿血液科,福建福州350001 [2]福建省血液病研究所/福建省血液病学重点实验室/福建医科大学附属协和医院血液科,福建福州350001

出　　处：《中国当代儿科杂志》2018年第6期470-474,共5页Chinese Journal of Contemporary Pediatrics

基　　金：福建省血液医学中心建设项目资助(闽政办(2017)4号);国家和福建省临床重点专科建设项目资助

摘　　要：目的了解儿童成熟B细胞非霍奇金淋巴瘤(B-NHL)的临床特征和治疗结局。方法 28例患儿均以CCCG-B-NHL?2010方案化疗,其中20例联合美罗华治疗,随访31(4~70)个月。回顾性分析患儿的临床特征,以Kaplan-Meier法进行生存分析,并且进行预后因素的单因素分析。结果 28例患儿中伯基特淋巴瘤(BL)17例(61%),弥漫大B细胞型淋巴瘤(DLBCL)8例(29%),3?例(11%)未能进一步分类。首发表现为颈部肿物的13例(46%),颌面部肿物10例(36%),肝脾大9例(32%),腹部包块5例(18%),突眼5例(18%)。LDH<500?U/L者14例,500~1?000?IU/L者3例,≥1?000?IU/L者11例。化疗2疗程后,21例完全缓解、7例部分缓解,截至随访末期,24例持续完全缓解、4例复发。2年无事件生存率为85.7±6.6%。骨髓活检提示骨髓浸润、LDH≥500?IU/L、骨髓肿瘤细胞>25%的成熟B-NHL患儿2年累积生存率较低。结论 CCCG-B-NHL?2010方案联合美罗华治疗儿童B-NHL疗效满意,骨髓活检发现的骨髓浸润与不良预后相关。Objective To study the clinical features and treatment outcome of children with mature B-cell nonHodgkin＇s lymphoma（B-NHL）. Methods A total of 28 previously untreated children with mature B-NHL were enrolled and given the chemotherapy regimen of CCCG-B-NHL-2010. Among them, 20 were given rituximab in addition to chemotherapy. The children were followed up for 31 months（ranged 4-70 months）. A retrospective analysis was performed for the clinical features of these children. The Kaplan-Meier method was used for survival analysis. A univariate analysis was performed to investigate the prognostic factors. Results Among the 28 children, 17（61%） had Burkitt lymphoma, 8（29%） had diffuse large B-cell lymphoma（DLBCL）, and 3（11%） had unclassifiable B-cell lymphoma. As for the initial symptom, 13（46%） had cervical mass, 10（36%） had maxillofacial mass, 9（32%） had hepatosplenomegaly, 5（18%） had abdominal mass, and 5（18%） had exophthalmos. Of all children, 14 had a lactate dehydrogenase（LDH） level of 〈500 IU/L, 3 had a level of 500-1 000 IU/L, and 11 had a level of ≥ 1 000 IU/L. After two courses of chemotherapy, 21 children achieved complete remission and 7 achieved partial remission. At the end of follow-up, 24 achieved continuous complete remission and 4 experienced recurrence. The 2-year event-free survival rate was（85.7±6.6）%. The children with bone marrow infiltration suggested by bone marrow biopsy, serum LDH ≥500 IU/L, and bone marrow tumor cells〉 25% had a low 2-year cumulative survival rate. Conclusions The CCCG-B-NHL 2010 chemotherapy regimen combined with rituximab has a satisfactory effect in the treatment of children with B-NHL. Bone marrow infiltration on bone marrow biopsy is associated with poor prognosis.

关 键 词：成熟B细胞非霍奇金淋巴瘤 美罗华 无事件生存率 预后 儿童 

分 类 号：R733.1[医药卫生—肿瘤]