Clinicopathological features analysis of monomorphic epitheliotropic intestinal T-cell lymphoma
10.3760/cma.j.cn101480-20211117-00095
- VernacularTitle:单形性嗜上皮性肠道T细胞淋巴瘤临床病理特征分析
- Author:
Hailing LIU
1
;
Xiaoying LOU
;
Yan HUANG
Author Information
1. 中山大学附属第六医院病理科,广州 510655
- Publication Type:Journal Article
- Keywords:
Monomorphic epitheliotropic intestinal T-cell lymphoma;
Pathological features;
Inflammatory bowel disease;
Diagnosis;
Differential diagnosis
- From:
Chinese Journal of Inflammatory Bowel Diseases
2022;06(4):323-329
- CountryChina
- Language:Chinese
-
Abstract:
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.