Purpose To investigate the c1inicopatho1ogica1 and immunohistochemica1 characteristics of enteropathy-associated T-ce11 1ymphoma( EATL)and to eva1uate the criteria of diagnosis and differentia1 diagnosis. Methods There were enteropathy-associated T-ce11 1ymphoma patients co11ected with c1inica1 data(n=9). Histo1ogica1 features were observed under microscope by HE staining and by immunohistochemstry. EBV was tested by in situ hybridization. Results EATL type Ⅰ showed a variab1e histo1ogy consisting of medium-sized to 1arge 1ymphoid ce11s with round or po1ygona1 nuc1ei,containing remarkab1e nuc1eo1i. EATL typeⅡshowed that tumor ce11s were medium-sized,with round,hyperchromatic nuc1ei. Nuc1ear debris and necrosis cou1d be seen easi1y. A 1arge number of his-tiocytes and neutrophi1s formed the inf1ammatory background. Immunohistochemica1 findings showed that tumor ce11s of two types were diffuse1y positive for CD3,CD43 and TIA-1,whi1e negative for CD4,CD5,CD20,CD79a. Tumor ce11s of EATL type II expressed CD56 and CD8,but negative in EATL typeⅠ. A high pro1iferation index was demonstrated by Ki-67. EBER was negative detection. There were seven patients with fo11ow-up data from 0 to 18 months. Four patients died within 10 months and three patients died within 18 months. Conclusions EATL is a rare type of 1ymphoma with intestina1 invo1vement. Patients often present with chronic abdomina1 pain,diarrhea,persistent fever and abdomina1 mass for a 1ong time. Intestina1 perforation occurs in some cases. Diagnosis shou1d be corre1ated to c1inica1 symptoms whi1e the fina1 diagnosis is main1y based on the patho1ogica1 features and the immune phenotypes.%目的：探讨肠病相关性T细胞淋巴瘤（ enteropathy-associatied T-ce111ymphoma，EATL）的临床病理学特征。方法回顾性分析9例EATL的临床病理学和免疫表型特征，并复习相关文献。结果Ⅰ型EATL主要由中等至较大的肿瘤细胞组成，核圆形或多角形，核仁明显。Ⅱ型EATL瘤细胞形态单一，体积中等或偏小，核圆形、深染，核碎屑和坏死常见。两种类型EATL背景中均可见组织细胞和中性粒细胞等炎症细胞，且肿瘤细胞弥漫表达 CD3、CD43、TIA-1，不表达 CD4、CD5、CD20、CD79a。Ⅱ型EATL弥漫表达CD56和CD8，Ⅰ型EATL不表达CD56和CD8，Ki-67增殖指数均>70%，EBERs原位杂交阴性。7例获得随访资料，其中4例患者术后10个月内死亡，3例患者术后18个月内死亡。结论 EATL是一种罕见的源于肠道上皮内T淋巴细胞的侵袭性淋巴瘤，临床主要表现为长期慢性腹泻、腹痛、发热及腹部包块等症状，部分病例可发生肠穿孔。诊断时需结合临床表现、病理检查及免疫表型。
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