Monomorphic Epitheliotropic Intestinal T-Cell Lymphoma: Clinicopathologic and Genetic Analysis of a Large Chinese Cohort.

Authors

Keya Li, Ran Wei, Mingzhen Su, Ruohong Shui, Hongfen Lu, Xiaoqiu Li, Xiongzeng Zhu, Xiaoyan Zhou, Baohua Yu

Published in

The American journal of surgical pathology. Oct 16, 2026. Epub Oct 16, 2026.

Abstract

Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) is a rare and aggressive T-cell lymphoma with incompletely characterized features. We analyzed 120 Chinese patients (88 males, 32 females; median age 58y), including 43% at stage I. All patients had gastrointestinal (GI) involvement, with 78% confined to the small intestine. The tumor cells were typically monomorphic and medium-sized, expressing CD3 (99%), CD7 (90%), CD8 (94%), CD56 (90%), and TIA-1 (97%). Low expression rates were noted for CD4 (9%) and CD5 (13%). CD20 was aberrantly expressed in 4% cases. TCRαβ expression was more common than TCRγδ (1.1:1). The next-generation sequencing (NGS) results showed that all cases had loss-of-function mutations in SETD2 (100%). Other frequently mutated genes included STAT5B (67%), JAK3 (50%), TP53 (33%), and KRAS (22%). All patients had detectable single nucleotide polymorphisms (SNPs). Copy number variations (CNVs) were detected in TSC2 (11%), CDH1 (5%), STK11 (5%), CDKN2B (5%), and SDHD (5%). In addition, novel fusions involving PML-RPH3A (5%), TP63-PRKAR2A (5%), MAD2L1-CD28 (5%), and DPP6-TP63 (5%), were identified. Among these patients, 89% underwent surgery, 83% received systemic chemotherapy, and 7% received stem cell transplantation (SCT). The median overall survival (OS) of 77 patients was 9 months. Age 58 years or older and Ki-67 >50% were significantly associated with poor prognosis in univariate analysis, while Ki-67 >50% and the presence of B symptoms were independent adverse prognostic factors in multivariate analysis. Our findings expand the molecular spectrum of MEITL and support precision medicine approaches for this therapy-resistant disease.

PMID:
42885229
Bibliographic data and abstract were imported from PubMed on 16 Oct 2026.

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