伴有11号染色体长臂异常的Burkitt样淋巴瘤临床病理分析并文献复习  

Clinicopathological analysis and literature review of Burkitt-like lymphoma with 11q aberration

作　　者：张洪兰[1] 杨春梅 杨聪颖[1] 张春芳[1] 聂艳红[1] 贺艳玲[1] 王倩 何万能 张昶[1] 陈昊[1] Zhang Honglan;Yang Chunmei;Yang Congying;Zhang Chunfang;Nie Yanhong;He Yanling;Wang Qian;He Wanneng;Zhang Chang;Chen Hao(Department of Pathology,The First People's Hospital of Lianyungang,Jiangsu Province,Lianyungang 222000,China;Department of Oncology,Dongying People's Hospital,Shandong Province,Dongying 257091,China)

机构地区：[1]江苏省连云港市第一人民医院病理科,连云港222000 [2]山东省东营市人民医院肿瘤科,东营257091

出　　处：《中国综合临床》2022年第3期222-227,共6页Clinical Medicine of China

摘　　要：目的探讨伴有11号染色体长臂(11q)异常的Burkitt样淋巴瘤(Burkitt-like lymphoma with 11q aberration,BLL-11q)临床病理特征、鉴别诊断、治疗及预后。方法回顾性分析连云港市第一人民医院2020年和2021年就诊的2例BLL-11q患者的临床表现、组织学形态、免疫表型和分子遗传学改变,并复习相关文献。结果2例患者均为右颈部包块,生长迅速,切除送检。Ann Arbor分期为ⅠA和ⅡA期。镜下淋巴结正常结构消失,代之以弥漫浸润性生长的中等大小的肿瘤细胞,细胞形态一致,"星空"现象明显,类似于Burkitt淋巴瘤。免疫表型:肿瘤细胞弥漫阳性表达CD20、CD79α、PAX5、CD10和Bcl-6,部分中等阳性表达C-MYC和MUM-1,CD3、Bcl-2、CD30和TDT均阴性,Ki-67阳性指数均>95%,EBER均阴性。荧光原位杂交检测显示MYC、Bcl2、Bcl6断裂,11q23.3扩增和11q24.3缺失。2例患者均行化疗,随访10~22个月,均获得完全缓解、无病生存。结论BLL-11q是一种罕见的生发中心B细胞性淋巴瘤,伴有第11号染色体长臂异常,且缺乏MYC基因重排,应与Burkitt淋巴瘤、弥漫性大B细胞淋巴瘤、B淋巴母细胞性淋巴瘤、伴有IRF4重排的大B细胞淋巴瘤、高级别B细胞淋巴瘤等鉴别,在形态学及免疫表型基础上诊断依靠基因学检测,可能有更好的预后。Objective To investigate the clinicopathological features,differential diagnosis,treatment and prognosis of Burkitt-like lymphoma with 11q aberration(BLL-11q).Methods The clinical manifestations,histological morphology,immunophenotype and molecular genetic changes of 2 cases of BLL-11q admitted to the department of pathology of The First People's Hospital of Lianyungang in 2020 and 2021 were analyzed retrospectively,and the relevant literatures were reviewed.Results Patients were found with right neck masses inadvertently and grew rapidly.They presented with localized disease with Ann Arbor stages IA and IIA.Microscopically,the normal structure of the lymph node disappeared and was replaced by a diffuse proliferation of lymphocytes,with consistent morphology and medium size.And the presence of"star-sky"phenomenon was obvious,the morphological characteristics were similar to Burkitt lymphoma.Immunophenotypically,tumor cells were diffusely positive for CD20,CD79α,PAX5,CD10 and Bcl-6,partly moderately positive for C-MYC and MUM-1,however,CD3,Bcl-2,CD30 and TDT were negative,Ki-67 positive index was more than 95%,and EBER was negative.FISH detection showed that MYC,Bcl-2,and Bcl-6 were negative.Both cases had the 11q23.3 gain and 11q24.3 loss.Both patients were treated with chemotherapy and followed up for 10-22 months,and achieved complete remission and disease-free survival.Conclusion BLL-11q is a rare germinal center B-cell lymphoma with abnormal long arm of chromosome 11 and lack of MYC gene rearrangement.It should be distinguished from Burkitt lymphoma,diffuse large B-cell lymphoma,B-lymphoblastic lymphoma,large B-cell lymphoma with IRF4 rearrangement and high-grade B-cell lymphoma.On the basis of morphology and immunophenotype,the diagnosis depends on genetic detection.There may be a better prognosis.

关 键 词：淋巴瘤 11号染色体长臂异常 MYC基因重排 预后 

分 类 号：R733.1[医药卫生—肿瘤]