Monomorphic Epitheliotropic Intestinal T-cell Lymphoma: A Study of Four Cases and Review of Literature.

Monomorphic Epitheliotropic Intestinal T-cell Lymphoma: A Study of Four Cases and Review of Literature.
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单形性上皮性肠 T 细胞淋巴瘤:四例研究及文献复习。

DOI:
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发表时间:
2020
影响因子:
0.8
通讯作者:
Sameer Al Diffalha
Sameer Al Diffalha
中科院分区:
医学4区
文献类型:
--
作者:
F. Fei;Vishnu V. B. Reddy;Chirag R Patel;D. Dhall;Goo Lee;Xiong Meng;Sameer Al Diffalha

文献摘要

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单形上皮性肠t细胞淋巴瘤(MEITL)是一种罕见的原发性、高度侵袭性的肠t细胞淋巴瘤,起源于上皮内淋巴细胞。MEITL以前被指定为II型肠病相关T细胞淋巴瘤(EATL)。与经典形式的EATL不同,MEITL与乳糜泻无关。MEITL的诊断非常具有挑战性,患者的临床预后非常差。在此,我们描述了一系列的四名患者诊断为MEITL确定了10年的机构回顾性审查。组织病理学检查显示,4例患者均有单一的中等大小细胞浸润肠黏膜,CD3、CD8和CD56均呈阳性。2例患者联合化疗;然而,这两名患者在诊断后的平均生存时间仅为7.5个月。本病例系列的目的是在现有文献的基础上强调MEITL患者的病理,诊断和临床过程。
Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) is a rare primary and highly aggressive intestinal T-cell lymphoma derived from intraepithelial lymphocytes. MEITL is previously designated as type II enteropathy-associated T cell lymphoma (EATL). Unlike to classic form of EATL, MEITL is not associated with celiac disease. The diagnosis of MEITL is very challenging and the clinical outcome of patients with MEITL is very poor. Herein we describe a series of four patients diagnosed with MEITL identified upon a 10-year institutional retrospective review. Histopathologic examination of these cases revealed monotonous population of medium sized cells infiltrating intestinal mucosa, positive for CD3, CD8 and CD56 in all four cases. Two patients had the combination chemotherapy; however, the average survival time was only 7.5 months for these two patients after diagnosis. The aim of the present case series is to highlight the pathology, diagnosis and clinical course of the patients with MEITL based on the current literature.