CD20^+外周T细胞淋巴瘤1例并文献复习  

Clinical pathological feature and molecular genetic analysis of CD20 positive peripheral T cell lymphoma and review literature

作　　者：李新霞[1] 古丽那尔.阿布拉江 马志萍[1] 张巍[1] 周晓燕[2] 盛伟琪[2] 李巧新[1] 

机构地区：[1]新疆医科大学第一附属医院病理科,乌鲁木齐830054 [2]复旦大学附属肿瘤医院病理科,上海230002

出　　处：《临床与实验病理学杂志》2013年第9期986-990,995,共6页Chinese Journal of Clinical and Experimental Pathology

基　　金：国家自然科学基金(81360352);新疆维吾尔自治区科技计划(201233142)

摘　　要：目的观察CD20+外周T细胞淋巴瘤的临床病理学特征、分子遗传学改变并复习文献,以提高对此罕见亚型的诊断及鉴别诊断。方法采用免疫组化EnVision两步法观察1例CD20+外周T细胞淋巴瘤的免疫表型,原位杂交法分析EB病毒感染,应用PCR技术检测IgH、T细胞受体(TCR)基因克隆性重排分析,同时结合文献报道的35例CD20+T细胞淋巴瘤与该病例分析其临床病理学特征和预后。结果本例及文献报道共36例,其中男性24例,女性6例,另6例性别不详,平均年龄58岁,中位年龄67岁,组织学类型为最常见非特指型外周T细胞淋巴瘤(22例)。免疫表型:肿瘤细胞强表达T细胞标记(CD2、CD3、CD5、CD8)及滤泡辅助T细胞标记(CD10、BCL-6、CXCL13、PD1),弱弥漫表达CD20、CD79a,不表达CD4、PAX5。瘤细胞间散在转化的大B免疫母细胞EBER阳性。PCR法检测TCR基因单克隆重排。患者接受R-CHOP治疗。结合文献:获得随访16例,随访时间2~16个月,其中9例死亡,中位生存时间为9个月,死亡与存活病例患者年龄差异无显著性,组织学亚型差异也无显著性。结论 CD20+外周T细胞淋巴瘤是一种罕见外周T细胞淋巴瘤的变异亚型,临床及病理特征可能会与B细胞淋巴瘤混淆,形态学观察、多种抗体的联合使用及TCR基因重排等分子遗传学研究可避免误诊,部分肿瘤免疫表型和血管免疫母细胞性T细胞淋巴瘤相似,提示二者可能是同一肿瘤的不同阶段或两者存在互相演进的关系。Purpose To observe the clinicopathologic and molecular genetic feature of CD20 positive peripheral T-cell lymphoma （CD20 ＋ PTCL） , and to improve its diagnosis and differentiated diagnosis ability. Methods The immunotype of a case of CD20 ＋ PTCL was observed by immunohistoehemistry. Detection of EBV-encoded RNAs （EBER） was performed by in situ hybridization （ISH）. Clonality gene rearrangement was conducted using PCR assays. Thirty-five cases of CD20 ＋ TCL previously reported in the lit- erature were reviewed. Results There were 36 cases in the literature including the present case, the male was 24 cases, the female was 6 cases, 6 cases were not performed. The mean age was 58 years and the median age was 67 years. Amongst the 36 reported ca- ses, lymphadenopathy was the commonest clinical manifestations. PTCL-U was the most common histological subtype （22 cases）. Im- munophenotype, neoplastic cells showed positive reactivity to CD2, CD3, CDS, CDS, CD10, PD1, BCL-6 and CXCL13. Positive hy- bridization signals for EBER was demonstrated that those EBV-positive cells were mostly the reactive transformed B-cells. Monoclonal T-cell proliferation were proved by the rearranged TCR gene detection and the patient received R-CHOP-based chemotherapy. Sixteen patients were followed up for 2 to 16 months. The median survival was 9 months. The difference between histological subtype and the survival rate was not statistically significant. Conclusion CD20 ＋ TCL may cause diagnostic difficulty, particularly in cases that clini- cally and pathologically mimicking B cell lymphoma （BCL）. Knowledge of the unusual phenomenon of CD20 expression in TCL, in conjunction with careful morphologic analysis, the use of a panel of antibodies, and molecular genetic studies, was important in avoi- ding a misdiagnosis of BCL. CD20 ＋ PTCL showed the similar or overlapped morphological and immunophenotypic features to those of AITL, possibly suggesting the presence of a potential relationship between these two types of

关 键 词：外周T细胞淋巴瘤 CD20阳性 免疫表型 原位杂交 基因重排 

分 类 号：R733[医药卫生—肿瘤]