作者：朱重梅，金杰畑，卢佳斌，黄丽云，陈跃琼

单位：中山大学肿瘤防治中心 病理科，广东 广州 510060

Authors:  Zhu Chongmei, Jin Jietian, Lu Jiabin, Huang Liyun, Chen Yueqiong

Unit:  Department of Pathology, Sun Yat-sen University Cancer Center, Guangzhou 510060, Guangdong，China

摘要：

目的 探讨单形性亲上皮性肠道T细胞淋巴瘤临床病理特点、免疫表型及预后。方法 收集中山大学肿瘤防治中心单形性亲上皮性肠道T细胞淋巴瘤共30例病史资料,组织经常规HE方法染色,光学显微镜下观察病理形态特点,通过免疫组化方法检测肿瘤细胞CD3、CD5、CD4、CD8、CD20、CD56、TIA1、granzyme B、perforin、Ki67蛋白表达情况,原位杂交方法检测EB病毒是否存在。结果 30例单形性亲上皮性肠道T细胞淋巴瘤发病年龄35～82岁,平均年龄52.2岁。男性23例,女性7例,男女比约为3:1。临床表现多数为腹痛,其次为消化道出血、体重减轻、肠梗阻或肠穿孔等,个别病例伴腹泻。病变部位以小肠最多见(24例),其次是结肠(6例)。镜下见瘤细胞形态单一,胞浆淡染,细胞核圆形,染色质细腻,核仁不明显。肿瘤表面可见溃疡形成,肠绒毛结构破坏,并可见"亲上皮现象",肿瘤性坏死罕见。免疫表型为CD3+、CD5-、CD4-/+、CD8+/-、CD56+/-、TIA1+、granzyme B+/-、perforin+/-,少数病例异常表达CD20,Ki67增殖指数40%～95%不等,原位杂交EBER阴性。结论 单形性亲上皮性肠道T细胞淋巴瘤具有独特的病理形态特点及免疫表型,但临床表现无特异性,病程发展快、预后差,早期易误诊漏诊,临床及病理医生均需提高对该病的认识,早诊早治以提高患者生存率。

关键词：单形性亲上皮性肠道T细胞淋巴瘤; 免疫组化; 临床病理特点

Abstract：

Objective To investigate the clinicopathological characteristics, immunophenotype and prognosis of monomorphic epitheliotropic intestinal T-cell lymphoma. Methods In this study, 30 patients with monomorphic epitheliotropic intestinal T-cell lymphoma were investigated retrospectively in our hospital. We utilized conventional HE staining to evaluate morphological features with an optical microscope. Immuno histochemistry staining including CD3, CD5, CD4, CD8, CD20, CD56, TIA1, granzyme B, perforin, Ki67 were performed to detect the protein expression, and in situ hybridization of EBERs to detect the presence of EB virus. Results In our cohort, the mean age at diagnosis was 52.2 (range from 35 to 82) years. There were 23 males and 7 females, and the male to female ratio was about 3: 1. Most of the clinical manifestations were abdominal pain, followed by gastrointestinal bleeding, weight loss, intestinal obstruction or intestinal perforation, etc., and rare cases with diarrhea. Lesion occurred most commonly in the small intestine (24 cases), followed by the colon (6 cases). The morphology features of the tumor cells involved uniform cell shape, pale cytoplasm, round nucleus, fine chromatin and the inconspicuous nucleoli. The tumor surface showed ulcer formation with the villous architecture distorted. There was usually florid infiltration of intestinal epithelium. Tumor necrosis was rare. Immune phenotypes were CD3+, CD5-, CD4-/+, CD8+/-, CD56+/-, TIA1+, granzyme B+/-, perforin+/-. And CD20 was abnormally expressed in some cases, Ki67 proliferation index ranged from 40% to 95%, and in situ hybridization of EBER was negative. Conclusions Monomorphic epitheliotropic intestinal T -cell lymphoma harbors unique morphological characteristics and immune phenotype, but the clinical manifestations are non-specific. Most cases develop rapidly, and the prognosis is poor. It is easy to be misdiagnosed and overlooked in the early stage. Both clinical doctors and pathologists need to improve the understanding of the disease, which can make the early diagnosis and early treatment possible to improve patients’ survival.

Key Words:  Monomorphic epitheliotropic intestinal T-cell lymphoma; Immunohistochemistry; Clinicopathological characteristics