Long-term disease control of monomorphic epitheliotrophic intestinal T-cell lymphoma (MEITL) with CNS involvement in a caucasian patient by allogeneic hematopoietic cell transplantation
Year: 2025
Type: article
Abstract: Monomorphic epitheliothrophic intestinal T-cell lymphoma (MEITL), formerly known as type II enteropathic T-cell lymphoma, is an aggressive form of peripheral T-cell lymphoma (PTCL) primarily originating from intraepithelial lymphocytes of the intestine [ 1 ]. Arising mostly in the small intestine with solitary or multifocal, often destructing lesions, it can disseminate in lymph nodes and non-intestinal organs including bone marrow and the central nervous system (CNS) [ 1 , 2 , 3 , 4 ]. It predominantly affects Asian and Hispanic populations but very rarely also Caucasians [ 1 , 5 ]. Generally, MEITL responds poorly to anthracycline-based lymphoma-typic induction chemotherapy, prompting investigators to explore more aggressive first-line treatment including high-dose therapy with autologous hematapoietic cell transplantation (auto-HCT). Although this strategy may be associated with better disease control, almost all patients will eventually relapse [ 6 , 7 ]. Accordingly, the prognosis of MEITL is dismal with median survival times around 12 months and only few long-term survivors [ 2 , 3 , 6 , 7 ]. The outcome is particularly poor if CNS involvement is present, a recent literature review identified only a single survivor among 12 cases reported [ 4 ]. Thus, expert recommendations advocate consolidation of MEITL responses by cellular immunotherapy through allogeneic hematopoietic cell transplantation (alloHCT) in analogy to other aggressive extranodal PTCL with similar biological behavior, such as hepatosplenic T-cell lymphoma [ 8 , 9 ] and acute/lymphoma-type adult T-cell leukemia/lymphoma [ 10 , 11 ], although the evidence basis for this is very poor.
Source: Bone Marrow Transplantation
Institutions: SLK-Kliniken Heilbronn, Heidelberg University
Language: English
Subfield: Pathology and Forensic Medicine
Field: Medicine
Domain: Health Sciences
Open Access status: hybrid
Estimated APC paid: $4,990
OpenAlex ID: W4416240506