ArticleHaematologica2023
Monomorphic epitheliotropic intestinal T-cell lymphoma comprises morphologic and genomic heterogeneity impacting outcome.
Article in Haematologica, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 50 papers, 1 of them a synthesis that pooled it.
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Who cites it
50 citing papers in PubMed, 1 synthesis or guideline pooled it, 67 citations in OpenAlex.
- [Current status and progress in the diagnosis and treatment of monomorphic epitheliotropic intestinal T-cell lymphoma].Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi · 2026Pooled it
- Article
- Intestinal epithelial SETD2 maintains gut microbial homeostasis to attenuate colitis.Clinical and translational medicine · 2026Article
- Genomic loss of MLH1/PMS2 loci defines a mismatch repair deficient subgroup in monomorphic epitheliotropic intestinal T-cell lymphoma.Blood cancer journal · 2026Article
- [Intestinal T-cell lymphomas and NK/T-cell lymphoproliferations-an overview].Pathologie (Heidelberg, Germany) · 2026Review
- Multiple Elective Surgeries and Cord Blood Transplantation for Multi-refractory Monomorphic Epitheliotropic Intestinal T-cell Lymphoma.Internal medicine (Tokyo, Japan) · 2026Article
- Duodenal Lymphomas: Comprehensive Evaluation of Endoscopic Features and Clinical Outcomes in a Tertiary Center.Diagnostics (Basel, Switzerland) · 2026Article
- Targeting signaling pathways in lymphoma: From molecular mechanisms to clinical breakthroughs.Chinese medical journal · 2026Review
- JAK Inhibitors in the Treatment of T-Cell Lymphomas: Current Evidence and Future Directions.Cancers · 2026Review
- Small bowel lymphoma presenting with frequent perforation: a single center experience focusing on the monomorphic epitheliotropic intestinal T-cell lymphoma.BMC gastroenterology · 2026Article
- Divergent molecular pathways drive monomorphic epitheliotropic and enteropathy-associated intestinal T-cell lymphoma.Leukemia · 2026Article
- Coexistence of monomorphic epitheliotropic intestinal T-cell lymphoma of the jejunum and bilateral adrenal pheochromocytomas: A case report.Radiology case reports · 2026Article
- Monomorphic epitheliotropic intestinal T-cell lymphoma presenting as acute small intestinal perforation: a case report and exploratory BCL-2-targeted therapy.Frontiers in medicine · 2026Article
- Long-term disease control of monomorphic epitheliotrophic intestinal T-cell lymphoma (MEITL) with CNS involvement in a caucasian patient by allogeneic hematopoietic cell transplantation.Bone marrow transplantation · 2026Article
- Case Report: Targeting the Achilles' heel of monomorphic epitheliotropic intestinal T-cell lymphoma.Frontiers in oncology · 2026Article
- Article
- The Significance of the Microenvironment in T/Nk-Cell Neoplasms.International journal of molecular sciences · 2025Review
- Cutaneous involvement as the presenting sign of monomorphic/epitheliotropic intestinal T-cell lymphoma (MEITL): A case report.JAAD case reports · 2025Article
- Leptomeningitis as Rare Secondary Dissemination in MEITL (Monomorphic Intestinal Epitheliotropic T-Cell Lymphoma).Life (Basel, Switzerland) · 2025Article
- Relapse of Monomorphic Epitheliotropic Intestinal T-Cell Lymphoma (MEITL) in a Pericardial Fluid.International journal of laboratory hematology · 2025Article
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Authors and funding
35 authors at 15 institutions in 5 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) is a rare aggressive T-cell lymphoma most reported in Asia. We performed a comprehensive clinical, pathological and genomic study of 71 European MEITL patients (36 males, 35 females, median age 67 years). The majority presented with gastrointestinal involvement and had emergency surgery, and 40% had stage IV disease. The tumors were morphologically classified into two groups: typical (58%) and atypical (i.e., non-monomorphic or with necrosis, angiotropism or starry-sky pattern) (42%), sharing a homogeneous immunophenotypic profile (CD3+ [98%] CD4- [94%] CD5- [97%] CD7+ [97%] CD8+ [90%] CD56+ [86%] CD103+ [80%] cytotoxic marker+ [98%]) with more frequent expression of TCRgd (50%) than TCRab (32%). MYC expression (30% of cases) partly reflecting MYC gene locus alterations, correlated with non-monomorphic cytology. Almost all cases (97%) harbored deleterious mutation(s) and/or deletion of the SETD2 gene and 90% had defective H3K36 trimethylation. Other frequently mutated genes were STAT5B (57%), JAK3 (50%), TP53 (35%), JAK1 (12.5%), BCOR and ATM (11%). Both TP53 mutations and MYC expression correlated with atypical morphology. The median overall survival (OS) of 63 patients (43/63 only received chemotherapy after initial surgery) was 7.8 months. Multivariate analysis found a strong negative impact on outcome of MYC expression, TP53 mutation, STAT5B mutation and poor performance status while aberrant B-cell marker expression (20% of cases) correlated with better survival. In conclusion, MEITL is an aggressive disease with resistance to conventional therapy, predominantly characterized by driver gene alterations deregulating histone methylation and JAK/STAT signaling and encompasses genetic and morphologic variants associated with very high clinical risk.
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