Analysis of clinicopathological features of 21 patients with enteropathy-associated T-cell lymphoma

目的 探讨肠病相关T细胞淋巴瘤(EATL)临床病理学特征及临床预后特点.方法 选取2008年1月至2015年5月诊断的肠道EATL 21例、结外自然杀伤/T细胞淋巴瘤(NKTCL) 11例及外周T细胞淋巴瘤(PTCL)6例.采用EnVision二步法检测肿瘤细胞免疫表型,检测EB病毒(EBV)编码小RNA(EBER),分析EBV感染情况.对部分病例进行临床随访.结果 21例EATL中,男性14例,女性7例,中位年龄55岁(40 ～ 79岁);发病部位为小肠15例,结肠4例,小肠及结肠多部位发病2例;3例为经典型EATL,1 8例为单形性EATL;肿瘤细胞CD3 ε、CD4、CD8、CD56、Granzyme B、TIA-1的阳性率分别为95.24％(20/21)、20.00％(3/15)、73.68％(14/19)、85.71％(18/21)、64.71％(11/17)、88.89％(16/18);EBER阳性率为0(0/21),明显低于NKTCL的100％(11/11).17例EATL患者有随访资料,中位生存时间为15个月(0～96个月).生存分析未发现三种淋巴瘤存在明显的预后差异(P=0.697).结论 EATL好发于中老年男性,好发于空肠.EATL的诊断需要临床病史、内窥镜表现、组织形态学改变、免疫表型检测及EBV检测等多方面信息综合分析,需与NKTCL及PTCL进行鉴别诊断.EATL发病率低且恶性度高,目前尚无有效的治疗方案.Objective To investigate the clinicopathological and prognosis features of enteropathy-associated T-cell lymphoma (EATL).Methods 21 cases of EATL,6 cases of peripheral T-cell lymphoma (PTCL) and 11 cases of natural-killer/T-cell lymphoma (NKTCL) were collected from January 2008 to May 2015.The immunophenotype of the tumor cell was tested by EnVision and as well as EBV-EBER for EB virus.Some patients were performed with follow-up data.Results 21 EATL patients included 14 males,7 females and the middle age was 55 years old (40-79 years old).15 patients affected the small bowel,4 cases affected colon,2 cases affected more than one site.18 cases were mono-morpholohic EATL while 3 cases were classical EATL.The expression rates of neoplastic cells for CD3ε,CD4,CD8,CD56,Granzyme B,TIA-1 were 95.24 ％ (20/21),20.00 ％ (3/15),73.68 ％ (14/19),85.71％ (18/21),64.71％ (11/17),88.89 ％ (16/18) respectively.The expression of EBER in EATL patients (0,0/21) was obviously lower than that in NKTCL patients (100 ％,11/11).17 EATL patients had follow-up data,and the middle survival time was 15 months.No different prognosis was found in the three kinds of T-NHL (P =0.697).Conclusions EATL usually occurs in elder male and jejunum.The diagnosis of EATL needs a lot of information,including clinical history,endoscopy,histomorphology,immunophenotype and EBV-EBER result.EATL has low mobidity and high malignancy,it still lacks impactful therapeutic regimen.中国科技核心期刊(ISTIC)5300-3032