摘要目的 探讨Ⅱ型肠病相关性T细胞淋巴瘤(Ⅱ型EATL)的临床病理特点、免疫表型、诊断、鉴别诊断以及预后.方法 对南京军区南京总医院病理科收集的14例Ⅱ型EATL患者临床资料、组织学、免疫组织化学进行观察分析并随访,结合文献讨论.结果 14例Ⅱ型EATL患者,男性12例、女性2例,中位年龄49岁,发生部位:空回肠(10例)、结肠(4例).临床主要表现为腹部肿块或腹痛、腹泻等肠道症状,可伴有发热、盗汗、消瘦等消耗性疾病的共同特点,均无麦麸过敏及乳糜泻的临床证据.镜下肿瘤细胞常呈透壁性浸润肠壁全层,瘤细胞核圆形,深染,胞质淡染,间质缺乏炎性背景,伴或不伴坏死；病变及其附近可见肠绒毛萎缩,隐窝增生,上皮内淋巴细胞增多.免疫表型瘤细胞表达CD3、CD43、CD8(14/14),CD56(11/14)、CD30(2/14)部分瘤细胞阳性,CD4、CD20、CD79a、髓过氧化物酶、EB病毒均阴性,Ki-67阳性指数高.EBER原位杂交均阴性.9例获得随访结果(6～36个月),其中7例在14个月内死亡.结论 Ⅱ型EATL患者,以肿瘤细胞累及肠道以至肠穿孔为特点,缺乏乳糜泻等肠病证据.诊断要结合临床表现和相关病史,并依据病理学特征及相关的免疫组织化学标志物综合判断而确诊.

abstractsObjective To study the clinicopathologic features,immunohistochemical findings,differential diagnosis and prognosis of type Ⅱ enteropathy-associated T-cell lymphoma (EATL).Methods Fourteen cases of type Ⅱ EATL encountered in Department of Pathology,Nanjing General Hospital were retrospectively reviewed.The clinical data,histologic features,immunohistochemical findings and follow-up information were analyzed,with literature review.Results There were altogether 12 males and 2 females.The median age of patient was 49 years.The sites of involvement included jejunum (10 cases) and ileum /colon (4 cases).The patients often presented with an abdominal mass,abdominal pain,diarrhea and constitutional symptoms such as fever,night sweating and cachexia.There was no clinical evidence of gluten-sensitive enteropathy.Histologically,the lymphoma cells showed full-thickness infiltration of the intestinal wall.They contained round hyperchromatic nuclei and pale cytoplasm.The stroma was minimally inflamed,with or without associated coagulative necrosis.A remarkable finding was the presence of villous atrophy,cryptal hyperplasia and intraepithelial lymphocytosis.Immunohistochemical study showed that the tumor cells expressed CD3,CD43 and CD8 (14/14).Some of them were also positive for CD56 (11/14)and CD30 (2/14).The staining for CD4,CD20,CD79a and myeloperoxidase was negative.A high proliferation index was demonstrated by Ki-67 immunostain.In-situ hybridization for EBER was negative.Follow-up data were available in 9 cases.The duration of follow-up ranged from 6 months to 36 months.Seven patients died within 14 months.Conclusions EATL is a rare type of lymphoma with intestinal involvement.Associated enteropathy is not demonstrated,in contrast to cases encountered in Nordic countries.A correct diagnosis requires evaluation of clinical manifestations,pathologic features and ancillary study results.