单形性嗜上皮性肠道T细胞淋巴瘤临床病理特征分析

Clinicopathological features analysis of monomorphic epitheliotropic intestinal T-cell lymphoma

目的探讨单形性嗜上皮性肠道T细胞淋巴瘤(MEITL)的临床病理特征、诊断及鉴别诊断。方法采用描述性病例系列研究方法。回顾性收集中山大学附属第六医院2016年1月至2021年7月收治的13例MEITL的临床资料,对部分病例补充免疫组化检测及EB病毒编码RNA检测(EBER)。分析患者的临床、内镜、影像学、病理特征。结果男性8例,女性5例。年龄34~65岁,中位年龄59岁。病变部位:小肠12例,结直肠7例,胃2例。肿瘤单发6例,其中小肠5例,结直肠1例;多发7例,分别是胃+小肠+结直肠2例,小肠+结直肠4例,空肠+回肠1例。5例患者内镜下可见黏膜充血水肿、小溃疡灶、息肉、肠壁狭窄和环周型肿物。11例CT显示胃壁或者肠壁增厚及肿块。肿瘤组织学显示,形态单一的小至中等大小淋巴样细胞弥漫浸润,伴显著嗜上皮性,背景无炎症细胞。免疫组化结果提示,CD56和T细胞胞质内抗原(TIA-1)均为阳性11例,CD4阳性1例,CD8阳性5例,CD4和CD8均阳性1例,CD4和CD8均阴性7例,1例异常表达CD20。12例行EBER检查,均为阴性。13例患者中,6例采用手术切除+化疗,3例采用单纯化疗,2例先采用手术切除但后续治疗不详,1例单纯手术切除,1例治疗方法不详。随访9例,时间1.0~16.1个月,7例死亡,2例存活,余4例失访。结论MEITL是罕见的肠道原发的侵袭性T细胞淋巴瘤,预后差。诊断需结合形态学、免疫组化和EBER,同时要与其他淋巴瘤和炎症性肠病进行鉴别诊断。

Objective To investigate the clinicopathological features,diagnosis and differential diagnosis of monomorphic epitheliotropic intestinal T-cell lymphoma(MEITL).Methods A descriptive case series study was performed.The clinical data of 13 patients with MEITL in the Sixth Affiliated Hospital of Sun Yat-sen University from January 2016 to July 2021 were collected retrospectively and immunohistochemical detection and detection of Epstein Barr virus encoded RNA(EBER)were supplemented in some patients.The clinical,endoscopic,imaging and pathological features were analyzed.Results The thirteen patients included 8 males and 5 females,with a median age of 59 years old(range from 34 to 65 years old).The tumors of 12 patients located in small intestine,7 in colorectum and 2 in stomach.There were 6 patients with solitary tumor,including 5 in small intestine and 1 in colorectum.There were 7 patients with multiple tumors,including 2 in stomach+small intestine+colorectum,4 in small intestine+colorectum and 1 in jejunum+ileum.The mucosal congestion and edema,small ulcer,polyps,intestinal wall stenosis and peripheral tumors were found in 5 patients by endoscope and thickening of gastric or intestinal wall and mass in 11 by CT.Tumor histology results showed that small to medium sized lymphoid cells with a single morphology were diffusely infiltrated,with the significant epitheliophilic characteristic,and there were no inflammatory cells in the background.The immunohistochemistry results showed that both CD56 and T-cell intracellular antigen(TIA-1)were positive in 11 patients,simple CD4 positive in 1,simple CD8 positive in 5,both CD4 and CD8 positive in 1,both CD4 and CD8 negative in 7 and aberrantly expression of the B-cell marker CD20 in 1.In situ hybridization for EBER was all negative(12/12).Among the 13 patients,6 patients were treated with surgical resection plus chemotherapy,3 patients were treated with simple chemotherapy,2 patients were treated with surgical resection at first but the follow-up treatment was unknown,1 patient was treated with surgical resection alone,and 1 patient was treated with unknown therapies.Nine patients were followed up for 1.0-16.1 months,7 died,2 survived and 4 were lost.Conclusions MEITL is a rare and primary invasive T-cell lymphoma of the intestine with poor prognosis,whose diagnosis is mainly based on the histological features,immunohistochemistry and EBER.This disease should be differentiated from other lymphomas and inflammatory bowel disease.