肠道T细胞淋巴瘤是一组异质性肿瘤。本文主要综述肠病型肠道T细胞淋巴瘤与不伴肠病的肠道T细胞淋巴瘤不同病变实体的病因学、临床病理特征、免疫表型、基因型以及诊断和鉴别诊断。二者均以肠道溃疡形成为特点 ,瘤细胞多为中等至大细胞性 ,表达T细胞标记 ,TCR基因重排。但前者多见于欧美国家 ,中老年患者居多 ,以腹痛、贫血和肠梗阻为主要症状 ,预后较好 ,与麦胶病相关 ,瘤细胞源自黏膜上皮内CD8(+)α/ β细胞毒性T细胞。后者多见于亚洲国家 ,青壮年多见 ,以贫血、便血、B症状、肠穿孔为主要临床表现 ,预后差 ;与EB病毒高度相关 ,肿瘤前体细胞尚不确定。肠道T细胞淋巴瘤的其他病变实体尚需进一步定义 Gut T-cell lymphoma is a heterogeneous group of tumors. This article reviews the etiology, clinicopathological features, immunophenotypes, genotypes, and diagnostic and differential diagnosis of different pathological types of enteropathy T-cell lymphoma and non-intestinal enteropathy T-cell lymphoma. Both are characterized by the formation of intestinal ulcers, tumor cells are mostly medium to large cell, the expression of T cell markers, TCR gene rearrangement. However, the former more common in Europe and the United States, the majority of middle-aged patients, with abdominal pain, anemia and intestinal obstruction as the main symptoms, the prognosis is good, and wheat glue disease, tumor cells derived from mucosal epithelial CD8 (+) alpha / beta cytotoxic T cell. The latter more common in Asian countries, more common in young adults, with anemia, blood in the stool, B symptoms, intestinal perforation as the main clinical manifestations, the prognosis is poor; highly correlated with Epstein-Barr virus precursor tumor cells is not yet determined. Other pathological entities of G-cell lymphoma need to be further defined