Cyclin D1免疫组织化学阴性套细胞淋巴瘤临床病理及分子遗传学特征分析  

Clinicopathological and molecular genetic features of cyclin D1-negative mantle cell lymphoma

作　　者：王冠男[1] 张岚[1] 李晨飞[1] 赵武干[1] 张丹丹[1] 张延平[1] 李文才[1] Wang Guannan;Zhang Lan;Li Chenfei;Zhao Wugan;Zhang Dandan;Zhang Yanping;Li Wencai(Department of Pathology,the First Affiliated Hospital of Zhengzhou University,Zhengzhou 450052,China)

机构地区：[1]郑州大学第一附属医院病理科,郑州450052

出　　处：《中华病理学杂志》2022年第4期296-300,共5页Chinese Journal of Pathology

摘　　要：目的探讨Cyclin D1免疫组织化学阴性套细胞淋巴瘤(mantle cell lymphoma,MCL)的临床病理学特征及分子遗传学改变。方法收集郑州大学第一附属医院病理科2016年1月至2021年7月确诊为Cyclin D1阴性MCL的病例,应用HE染色、免疫组织化学及荧光原位杂交(FISH)分析其病理学特点及分子遗传学特点,并收集临床资料。结果收集到MCL共212例,其中Cyclin D1阴性的MCL共5例(5/212,2.4%)。临床特征方面,5例Cyclin D1阴性MCL病例中,男性3例,女性2例,中位年龄64岁(年龄范围59~70岁)。病变部位均位于淋巴结。5例均无B症状,4例可见骨髓累及(4/5)。病理学特征方面,4例为经典型,1例为多形性变型。5例Cyclin D1均阴性(有阳性对照且重复2次免疫组织化学染色),但均表达SOX11(5/5),4例CD5阳性(4/5),5例均不表达CD10和bcl-6,1例弱表达CD23(1/5)。分子遗传学改变方面,经FISH检测3例见CCND1易位,1例见CCND2易位,5例均未见CCND3易位。结论Cyclin D1阴性MCL非常少见,需综合组织学形态、免疫表型及基因检测综合诊断,有时与其他小细胞型B细胞淋巴瘤鉴别诊断困难。常规开展CCND1/CCND2/CCND3基因易位检测,并结合SOX11表达是此类少见疾病诊断及鉴别诊断有价值的参考依据。Objective To investigate the clinicopathological features and molecular genetics of cyclin D1-negative mantle cell lymphoma(MCL).Methods The clinicopathological features and molecular genetics of CyclinD1-negative MCL diagnosed between January 2016 and July 2021 at the First Affiliated Hospital of Zhengzhou University were analyzed using immunohistochemistry and fluorescence in situ hybridization.Clinical information was collected and analyzed.Results A total of five Cyclin D1-negative MCL cases from all 212 MCL patients(5/212,2.4%)were included.There were three male and two female patients,age ranged from 59 to 70 years(median 64 years).All patients presented with nodal lesions.None of the patients had B symptoms but four had bone marrow involvement.Histopathologically,four cases were classic MCL and one case was pleomorphic variant type.All five cases were negative for Cyclin D1 but SOX-11 were positive in all cases.CD5 was positive in four cases and one case was weakly positive for CD23.CD10 and bcl-6 were negative in all cases.CCND1 translocation was identified in three cases and CCND2 translocation in one case by FISH analysis.However,CCND3 translocations were not found in the five cases.Conclusions Cyclin D1-negative MCL are uncommon,its accurate diagnosis needs combined analysis with morphologic and immunophenotypic characteristics and genetic changes.It may be particularly difficult to distinguish from other small cell type B cell lymphomas.FISH analyses for CCND1/CCND2/CCND3 translocations and immunohistochemistry for SOX-11 are helpful to resolve such a difficult distinction.

关 键 词：淋巴瘤 病理学 临床 免疫组织化学 

分 类 号：R733.1[医药卫生—肿瘤]