Objective  To retrospectively investigate clinicopathological characteristics, survival outcomes, and prognostic factors in patients with monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL).

Methods  The clinical data of patients diagnosed with MEITL at Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, from January 2013 to October 2024 were collected. A retrospective analysis was conducted on the clinical manifestations, pathological features, treatment regimens, and prognosis.

Results  Among the 31 patients with MEITL, the median age was 59 years (range, 26–74 years), with a male-to-female ratio of 2.44∶1. The most common symptoms were abdominal pain (77.4%), diarrhea(19.4%), and fever (19.4%). Extranodal involvement sites included the small intestine (83.9%), colon (22.6%), bone marrow (19.4%), stomach (9.7%), lungs (9.7%), and eyes (3.2%). Immunohistochemistry predominantly revealed a CD3⁺CD8⁺CD56⁺ phenotype, with all cases beingEpstein-Barr virus-encoded RNA-negative. The median overall survival was 9.4 months, with a one-year survival rate of 35.5%. Multivariate analysis identified an Eastern Cooperative Oncology Group score ≥2 (hazard ratio HR=3.07) and no response to initial treatment (HR=3.90) as independent poor prognostic factors for OS. The response rate in the surgery combined with chemotherapy group (42.1%) was higher than that in the chemotherapy-alone group (8.3%); however, this did not result in a significant improvement in long-term survival.

Conclusions  MEITL is highly aggressive and associated with a poor prognosis. Novel targeted therapies and intensive chemotherapy combined with transplantation strategies should be explored to improve survival outcomes.