Abstract

Abstract Background: Monomorphic Epitheliotropic Intestinal T-cell Lymphoma (MEITL) is a rare extranodal intestinal PTCL. It is an aggressive disease with an overall poor prognosis. However, the disease presents a diverse spectrum of survival outcomes. We conducted this study to develop a provisional MEITL prognostic score (MPS) for this rare disease. Methods: We used our updated MEITL database, which contains retrospective data on 218 cases. Such data included demographics such as sex, age, and race. It also included disease presentation symptoms, duration of symptoms before diagnosis, site(s) of the disease, stage, blood counts, disease immune and molecular phenotype, types of treatment, quality of response, and survival outcomes. Out of the 218 cases, only 140 cases had complete survival and outcomes data, the sample chosen for this study. Cox proportional-hazards model and Log-rank tests were used to assess the influence of clinicopathologic factors on overall survival (OS). Due to the relatively small training cohort, we included factors that statistically impacted OS as well as factors that numerically trended that way. Results: The median OS of the cohort was 11 months. The following dichotomous variables were identified as impactful prognostic factors in this cohort: Bone marrow involvement (9 vs. 14 months), Non-biclonal TCR status (10 vs. 17.5 months), CD56+ (10 vs. 26 months), Ki67>70% (4 vs. 8 months), cMyc expression >15% (7 vs. 12 months). A prognostic model was devised using these variables to identify different levels of risk. Each variable was assigned a score of 1 when present, except for cMyc, which was assigned 2 points for having the highest hazard ratio among all the variables. In this exploratory cohort, low risk was assigned a score of 0, intermediate risk a score of 1-2, and high risk a score of 3-6. This prognostic score system led to our cohort's most optimal risk discriminatory model, where low, intermediate, and high risk had a median OS of 26, 12, and 4 months, respectively (p=0.002). Conclusion: This MPS is a promising new tool for risk-stratifying patients with MEITL. However, it still needs prospective validation. Citation Format: Philip A. Haddad, Dinesh Keerty, Neelakanta Dadi. Provisional prognostic score for monomorphic epitheliotropic T-Cell lymphoma. [abstract]. In: Proceedings of the American Association for Cancer Research Annual Meeting 2023; Part 1 (Regular and Invited Abstracts); 2023 Apr 14-19; Orlando, FL. Philadelphia (PA): AACR; Cancer Res 2023;83(7_Suppl):Abstract nr 4408.

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