Advanced Case of Monomorphic Epitheliotropic Intestinal T-Cell Lymphoma

Purpose: Monomorphic Epitheliotropic Intestinal T-Cell Lymphoma (MEITL) is a very rare and highly aggressive peripheral T-cell lymphoma with poor prognosis and for which there is no standard treatment. Treatment options for patients patients with relapsed/refractory disease are scarce and the choice of an appropriate rescue still represents an unmet need.

Methods: Here, we report the case of a 65-year-old woman affected by MEITL, progressing after initial treatment with an anthracycline-based chemotherapy and surgery, who received single-agent PEG-asparaginase salvage therapy at our institution.

Results: PEG-asparaginase single-agent rescue proved to be rapidly effective in controlling the disease and its associated paraneoplastic features. Nevertheless, toxicity was high and the patient died due to a treatment-related complication.

Conclusion: The case we described brings new evidences on the effectiveness of PEG-asparaginase therapy in MEITL patients. Whether PEG-asparaginase should be included in the treatment course of MEITL patients could be the subject of future studies.

Keywords: Extranodal lymphomas; Gastrointestinal lymphomas; Monomorphic epitheliotropic intestinal T cell lymphoma; PEG-asparaginase; T cell lymphomas.