伴11号染色体长臂异常的儿童成熟B细胞淋巴瘤11例临床研究  

Clinical study of mature B-cell lymphoma in 11 children with chromosome 11 long-arm abnormalities

作　　者：张楠[1] 段彦龙[1] 周春菊[2] 金玲[1] 杨菁[1] 黄爽[1] 张梦[1] 李楠[1] Zhang Nan;Duan Yanlong;Zhou Chunju;Jin Ling;Yang Jing;Huang Shuang;Zhang Meng;Li Nan(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;Medical Oncology Department,Pediatric Oncology Center,Beijing Children’s Hospital,Capital Medical University,National Center for Children’s Health,Department of Pathology)

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]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

出　　处：《中华血液学杂志》2023年第11期924-929,共6页Chinese Journal of Hematology

摘　　要：目的探讨伴11号染色体长臂(11q)异常的儿童成熟B细胞淋巴瘤(MBCL)的临床特征及预后。方法回顾性分析2018年12月至2023年2月首都医科大学附属北京儿童医院收治的11例伴11q异常的MBCL患儿的临床资料。结果11例儿童MBCL患者中男9例,女2例,中位年龄9(2~13)岁,中位病程1.8(0.5~24)个月。临床表现为颈部淋巴结肿大4例,鼻塞、打鼾4例,腹部疼痛2例,呼吸困难1例。病理形态呈伯基特淋巴瘤样7例,滤泡性淋巴瘤样2例,高级别B细胞淋巴瘤样2例。所有患者均无中枢神经系统、骨髓累及,B超及PET/CT等影像学评估未见广泛转移,1例有巨大瘤灶。修订国际儿童非霍奇金淋巴瘤分期系统(IPNHLSS)Ⅱ期4例,Ⅲ期5例,Ⅳ期2例。11q探针检测显示,5例11q增益,3例11q缺失,3例增益和缺失同时存在。FISH显示3例患者C-MYC基因阳性,伴11q异常的高级别B细胞淋巴瘤8例,伴11q异常的伯基特淋巴瘤3例。根据国家卫生健康委员会2019版儿童侵袭性成熟B细胞淋巴瘤诊疗规范,A组化疗1例、B组2例、C组8例,早期评估疗效均完全缓解;B组及C组于中期评估后降低化疗强度,2例化疗中,其余9例中位随访32(6~45)个月均无事件生存。结论伴11q异常的儿童MBCL发病率低,临床症状轻、进展慢,行FISH检测存在11q异常,无MYC、BCL2、BCL6重排为其重要诊断要点,降低化疗强度预后良好。Objective To explore the clinical,pathological,diagnostic,treatment,and prognostic features of children with mature B-cell lymphoma(MBCL).Methods This retrospective study included pediatric patients with MBCL with chromosome 11 long-arm abnormalities who were diagnosed and treated at our hospital from December 2018 to February 2023.Results Among the 11 pediatric patients with MBCL,nine were male and two were female,with a median age of 9(2-13)years and a median disease course of 1.8(0.5-24)months.The clinical manifestations were cervical lymph node enlargement in four patients,nasal congestion and snoring in four patients,abdominal pain in two patients,and difficulty breathing in one patient.There were seven cases of Burkitt’s lymphoma,two of follicular lymphoma,and two of advanced B-cell lymphoma according to the pathological morphology examination.No patients had central nervous system or bone marrow involvement,and no extensive metastasis was observed on B-ultrasound or positron emission tomography-computed tomography(PET/CT).One patient had a huge tumor lesion.The Revised International Pediatric Non-Hodgkin Lymphoma Staging System classified four patients as stageⅡ,five as stageⅢ,and two as stageⅣ.11q probe detection showed five cases of 11q gain,three of 11q loss,and three of both gain and loss.FISH showed positive MYC expression in three patients,including eight with advanced B-cell lymphoma with 11q abnormalities and three with Burkitt’s lymphoma with 11q abnormalities.According to the 2019 edition of the National Health Commission’s diagnostic and treatment guidelines for invasive MBCL in children,one patient was classified as Group A,two as Group B,and eight as Group C.Early evaluation of the efficacy showed complete remission.After mid-term evaluation,the intensity of chemotherapy was reduced in Group B and Group C.Among two cases of chemotherapy,the remaining nine cases had a median follow-up of 32(6-45)months,and none had event-related survival.Conclusion The incidence of MBCL with 11q abn

关 键 词：B细胞淋巴瘤 11号染色体 儿童 临床 治疗 

分 类 号：R733.1[医药卫生—肿瘤]