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We provide an overview of recent advances in the diagnosis and classification of intestinal T- and NK-cell lymphomas and lymphoproliferative disorders that primarily involve the gastrointestinal (GI) tract. It should be mentioned that systemic T- and NK-cell lymphomas can present initially in the GI tract but are not the focus of this review. Here, we focus on the clinical and pathologic findings of enteropathy-associated T-cell lymphoma, monomorphic epitheliotropic intestinal T-cell lymphoma, intestinal T-cell lymphoma not otherwise specified, indolent T-cell lymphoproliferative disorder of the GI tract and indolent NK-cell lymphoproliferative disorder of the GI tract (previously known as NK-cell enteropathy).

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Introduction: Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) is a rare, aggressive subtype of primary gastrointestinal T-cell lymphoma. Owing to the absence of symptoms characteristic of MEITL, diagnosis can be challenging, and the low response rate to conventional chemotherapy leads to an abysmal prognosis. This study aimed to define the clinicopathologic characteristics of MEITL in Korea, evaluate the clinical outcomes of intensive chemotherapy with and without hematopoietic stem cell transplantation (HSCT), and explore prognostic factors.

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Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL), previously referred to as enteropathy-associated T-cell lymphoma (EATL) type II, is a rare type of intestinal extranodal T-cell lymphoma that arises from intraepithelial T-lymphocytes of the intestinal mucosa. Here, we report a case of MEITL with an unusual localization in the ileocecal region complicated by an adjacent abscess and perforation of colon ascendens in a 65-year-old male. The patient was admitted to the hospital with acute abdominal pain.

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Monomorphic Epitheliotropic Intestinal T-Cell Lymphoma of the Duodenum.

ACG Case Rep J

November 2024

Department of Medicine, Division of Gastroenterology & Hepatology, NYU Langone Health, New York, NY.

Monomorphic epitheliotropic intestinal T-cell lymphomas (MEITLs) are rare neoplasms that carry a poor prognosis. MEITLs originating in the duodenum are uncommon. There are only 3 published case reports of primary duodenal MEITLs.

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