Multiple lesions of gastrointestinal tract invasion by monomorphic epitheliotropic intestinal T-cell lymphoma, accompanied by duodenal and intestinal enteropathy-like lesions and microscopic lymphocytic proctocolitis: a case series

Abstract

BackgroundIn East Asia, monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL), previously known as type II enteropathy-associated T-cell lymphoma (EATL), occurs more frequently than type I EATL, and coeliac disease is rare.Case presentationHere we present four cases of MEITL in Japanese patients, including the endoscopic and pathological findings of their duodenal and colorectal lesions. Tumor specimens obtained from duodenal, intestinal, and colorectal biopsies in all four patients showed a diffuse intramucosal infiltration of small to/or medium-sized lymphoma cells and numerous atypical intraepithelial lymphocytes (IELs). These cells were immunohistologically positive for CD103, CD3, CD7, CD8, CD56, and T-cell intracellular antigen-1. Upper and lower gastrointestinal and antegrade double-balloon endoscopy revealed foci of edematous mucosa, with or without villous atrophy, in the non-neoplastic mucosa. Histological studies demonstrated duodenal and intestinal enteropathy-like lesions as well as microscopic (lymphocytic) proctocolitis with increased CD3- and CD8-positive and CD56-negative T-IELs in all four patients. The clinicopathological findings of the non-neoplastic lesions were similar to those characteristic of coeliac disease, suggesting that variants of coeliac disease may be present in the prodromal lesions of MEITL.ConclusionsOur study supports the need for random gastrointestinal biopsies to determine tumor spread, the features of MEITL in the particular patients, and the presence of prodromal non-neoplastic lesions.

Monomorphic epitheliotropic intestinal T‐cell lymphoma in pleural effusion: A case report

“…EATL Type I is associated with celiac disease and is more frequently encountered in Europe and America. Conversely, EATL type II is not related with typical celiac disease, but with similar forms of colitis 7 and are more frequent in East Asia . Morphology and immunophenotype assist in distinguishing the two different subtypes of EATL.…”

Monomorphic Epitheliotropic Intestinal T-Cell Lymphoma With Extraintestinal Areas of Peripheral T-Cell Lymphoma Involvement

“…Inflammatory bowel disease, indolent T-cell lymphoproliferative disease, EATL, and intestinal natural killer (NK)/T-cell lymphoma can be the differentials of MEITL [ 5 ]. Although there is no association between MEITL and celiac disease, recent reports have suggested some cases of MEITL to be preceded by a variant of celiac disease [ 6 ].…”

A unique case of mixed phenotype acute leukemia with t(9;22)(q34.1;q11.2);BCR-ABL1 sarcoma with epitheliotropism mimicking intestinal T cell lymphoma

“…indolent T cell lymphoproliferative disorders of the gastrointestinal tract [21][22][23][24]. Clinically, these T cell lymphomas share similar presentations with gastrointestinal symptoms such as diarrhea, abdominal discomfort, food intolerances, and weight loss [21][22][23][24]. Epitheliotropism has not been described in literature as a histologic feature of intestinal presentations of myeloid sarcomas.…”