伴IRF4重排的大B细胞淋巴瘤临床病理及分子遗传学特征  

Clinicopathological features and molecular genetics of large B-cell lymphoma with IRF4 rearrangement

作　　者：袁玲玲[1] 刘昕悦 姚莉 刘文文 童涵懋 汤显斌[1] YUAN Ling-ling;LIU Xin-yue;YAO Li;LIU Wen-wen;TONG Han-mao;TANG Xian-bin(Department of Pathology,Taihe Hospital,Hubei University of Medicine,Shiyan 442000,China)

机构地区：[1]湖北省十堰市太和医院(湖北医药学院附属太和医院)病理科,湖北十堰442000

出　　处：《诊断病理学杂志》2023年第7期669-672,共4页Chinese Journal of Diagnostic Pathology

摘　　要：目的探讨伴IRF4重排的大B细胞淋巴瘤(LBCL-IRF4)临床病理及分子遗传学特征。方法收集湖北省十堰市太和医院(湖北医药学院附属太和医院)2017-12—2020-12诊断的2例LBCL-IRF4,采用HE染色、免疫组化及荧光原位杂交的方法,观察组织学、免疫表型及分子遗传学特征,并结合文献进行复习。结果2例患者中男女各1例,年龄分别为11岁、7岁,发病部位分别在颈部淋巴结(例1)和扁桃体(例2);形态上,例1膨胀性生长构型;例2弥漫性浸润构型;免疫组化示肿瘤细胞表达全B细胞标记物,2例均弥漫表达CD10、Bcl-6、MUM1及Bcl-2,Ki-67增殖指数均大于80%。2例均出现IRF4基因断裂重排。结论LBCL-IRF4是一种少见并具有独特的临床病理和分子遗传学特征的大B细胞淋巴瘤,好发于儿童和年轻人,主要累及Waldeyer环或头颈部淋巴结,治疗后预后较好。Objective To investigate the clinicopathologic and molecular genetic characteristics of large B-cell lymphoma with IRF4 rearrangement(LBCL-IRF4).Methods Two cases of large B-cell lymphoma with IRF4 rearrangement diagnosed in Taihe Hospital of Shiyan City,Hubei Province from December 2017 to December 2020 were collected.HE staining,immunohistochemistry and fluorescence in situ hybridization were used to observe the histological,immunophenotype and molecular genetic characteristics,and the literature was reviewed.Results The two patients,one male and one female,aged 11 years and 7 years,respectively.The sites of onset were cervical lymph node(case 1)and tonsil(case 2),Morphologically,an expansive growth configuration in case 1 and diffuse infiltration configuration in case 2.Immunohistochemistry showed that the tumor cells expressed whole B cell markers,CD10,Bcl-6,MUM1 and Bcl-2 in both cases,and the proliferation index of Ki-67 was more than 80%.IRF4 gene breakage and rearrangement were observed in both cases.Conclusion Large B-cell lymphoma with IRF4 rearrangement is a rare type of large B-cell lymphoma with unique clinicopathological and molecular genetic characteristics.It most commonly occurs in children and young adults,mainly involving Waldeyer ring or head and neck lymph nodes,and has a good prognosis after treatment.

关 键 词：伴IRF4重排的大B细胞淋巴瘤 免疫组化 荧光原位杂交 

分 类 号：R735.2[医药卫生—肿瘤]