Monomorphic epitheliotropic intestinal T-cell lymphoma presenting as acute small intestinal perforation: a case report and exploratory BCL-2–targeted therapy - Report - MDSpire

Monomorphic epitheliotropic intestinal T-cell lymphoma presenting as acute small intestinal perforation: a case report and exploratory BCL-2–targeted therapy

  • By

  • Meijuan He

  • Yao Zhou

  • Jia Yao

  • Qiu Zhang

  • Wenjing Xu

  • Xiaoping Ye

  • Yihan Cai

  • Zefa Liu

  • June 5, 2026

  • 0 min

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Clinical Report: Acute Small Intestinal Perforation and MEITL Case Study

Overview

This report presents a rare case of monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) that manifested as acute gastrointestinal perforation. The patient achieved complete metabolic remission following a novel treatment regimen combining a BCL-2 inhibitor with CHOP chemotherapy.

Background

Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) is a rare and aggressive lymphoma often presenting with acute complications such as intestinal perforation. The prognosis for MEITL is poor, with a median overall survival of only 7–10 months, largely due to resistance to conventional chemotherapy. Understanding its clinical presentation and exploring novel therapeutic strategies is crucial for improving patient outcomes.

Data Highlights

Emergency surgery revealed MEITL, initially staged as Lugano stage I. The patient experienced a recurrence after 9 months, confirmed via pathology. Following treatment with Venetoclax and CHOP, the patient achieved complete metabolic remission.

Key Findings

  • MEITL is characterized by a distinct immunophenotype (CD3+ CD8+ CD56+).
  • High expression of Bcl-2 in MEITL suggests it may be a therapeutic target.
  • The patient initially presented with acute abdominal symptoms leading to emergency surgery.
  • After a 9-month untreated interval, the patient experienced disease relapse.
  • Combination therapy with Venetoclax and CHOP resulted in complete metabolic remission.

Clinical Implications

The case highlights the diagnostic challenges associated with MEITL and the potential role of BCL-2 inhibition in treatment. Clinicians should consider the aggressive nature of MEITL and the need for timely intervention to manage complications effectively.

Conclusion

This case underscores the importance of recognizing MEITL as a surgical emergency and suggests that BCL-2 targeted therapy may offer a promising avenue for treatment. Further validation in larger studies is warranted.

Related Resources & Content

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  2. Bone Marrow Transplantation -- Sustained Disease Management of Monomorphic Epitheliotropic Intestinal T-Cell Lymphoma with Central Nervous System Involvement in a Caucasian Individual Following Allogeneic Hematopoietic Cell Transplantation
  3. Peripheral T- and natural killer-cell lymphomas: ESMO-EHA Clinical Practice Guideline for diagnosis, treatment and follow-up
  4. Peripheral T-Cell Non-Hodgkin Lymphoma Treatment (PDQ®) - PDQ Cancer Information Summaries
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  7. The 5th edition of the World Health Organization Classification of Haematolymphoid Tumours: Lymphoid Neoplasms - PMC
  8. Clinicopathological and molecular genetic alterations in monomorphic–epitheliotropic intestinal T-cell lymphoma of the small intestine
  9. Hematopoietic stem cell transplantation to improve prognosis in aggressive monomorphic epitheliotropic intestinal T-cell lymphoma - PMC
  10. Peripheral T- and natural killer-cell lymphomas: ESMO-EHA Clinical Practice Guideline for diagnosis, treatment and follow-up - PubMed
  11. Peripheral T-Cell Non-Hodgkin Lymphoma Treatment (PDQ®) - PDQ Cancer Information Summaries - NCBI Bookshelf
  12. Venetoclax Shows Low Therapeutic Activity in BCL2-Positive Relapsed/Refractory Peripheral T-Cell Lymphoma: A Phase 2 Study of the Fondazione Italiana Linfomi - PMC

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