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