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An uncommon mature T-cell lymphoma of intraepithelial lymphocytes. It usually arises from the small intestine, most commonly the jejunum or ileum. Other less frequent primary anatomic sites include the duodenum, stomach, colon, or outside the gastrointestinal tract. It is seen with increased frequency in regions with a high prevalence of celiac disease.
No approved treatments are currently available for enteropathy-associated T-cell lymphoma. An additional 1 compound holds orphan drug designation.
While no drugs are FDA-approved specifically for enteropathy-associated T-cell lymphoma, some of the following designated compounds may be used off-label in clinical practice. Treatment decisions should be made in consultation with a specialist familiar with this condition.
The following drugs have received orphan drug designation from the FDA for enteropathy-associated T-cell lymphoma. Orphan designation reflects regulatory interest and does not indicate approval for treatment.
Brand Name | Generic Name | Sponsor |
|---|
10 clinical trials registered, 4 recruiting. Interventions under study include drug therapy, other interventions, biologic therapy, and procedural interventions. Pipeline includes 2 PHASE2, 6 PHASE1, 1 NA. Research is sponsored by a mix of industry and academic institutions.
NCT ID | Title | Phase | Sponsor | Status |
|---|---|---|---|---|
[NCT07055477](https://clinicaltrials.gov/study/NCT07055477) |
Data assembled from 5 of 12 sources · Last updated Sep 19, 2026, 2:57 PM UTC
European rare disease database
Genetic and Rare Diseases Info Center
Designated
Exclusivity End |
|---|
Designation Status |
|---|
brentuximab vedotin | brentuximab vedotin | Seattle Genetics, Inc. | 2016 | — | Withdrawn |
10 trials found
A Phase I Trial Anti-CC Chemokine Receptor 4 Chimeric Antigen Receptor T Cells (CCR4 CAR T Cells) for CCR4 Expressing T-cell Malignancies Including Peripheral T-cell Non-Hodgkin Lymphoma (PTCL) and Cutaneous T-cell Non-Hodgkin Lymphoma (CTCL) |
PHASE1 |
National Cancer Institute (NCI) |
RECRUITING |
[NCT05978141](https://clinicaltrials.gov/study/NCT05978141) | A Registry for People With T-cell Lymphoma | — | Memorial Sloan Kettering Cancer Center | RECRUITING |
[NCT05475925](https://clinicaltrials.gov/study/NCT05475925) | A Study of DR-01 in Subjects With Large Granular Lymphocytic Leukemia or Cytotoxic Lymphomas | PHASE1 | Dren Bio | RECRUITING |
[NCT04803201](https://clinicaltrials.gov/study/NCT04803201) | Testing the Addition of Duvelisib or CC-486 to the Usual Treatment for Peripheral T-Cell Lymphoma | PHASE2 | Alliance for Clinical Trials in Oncology | RECRUITING |
45 publications have been identified in PubMed for enteropathy-associated T-cell lymphoma. Research spans Case Report / Case Series (38%), Review / Meta-Analysis (29%), and Basic Science / Preclinical (13%).
Research Type | Count | % of Total |
|---|---|---|
Patient case studies | 17 | 38% |
Research summaries | 13 | 29% |
Laboratory research | 6 | 13% |
Disease patterns and progression | 6 | 13% |
Other research | 2 | 4% |
Clinical study results | 1 | 2% |
Vallois D (2026). [PMID: 41057685](https://pubmed.ncbi.nlm.nih.gov/41057685/). *Leukemia*. [Basic Science / Preclinical]
Al-Toma A (2026). [PMID: 41831197](https://pubmed.ncbi.nlm.nih.gov/41831197/). *United European Gastroenterol J*. [Review / Meta-Analysis]
Kojima K (2026). [PMID: 41913572](https://pubmed.ncbi.nlm.nih.gov/41913572/). *Vet Pathol*. [Basic Science / Preclinical]
Boughanmi F (2026). [PMID: 42057140](https://pubmed.ncbi.nlm.nih.gov/42057140/). *J Med Case Rep*. [Case Report / Case Series]
Li MH (2026). [PMID: 41551832](https://pubmed.ncbi.nlm.nih.gov/41551832/). *World J Gastroenterol*. [Review / Meta-Analysis]
Butler K (2026). [PMID: 41782401](https://pubmed.ncbi.nlm.nih.gov/41782401/). *Curr Opin Gastroenterol*. [Review / Meta-Analysis]
Gao Y (2026). [PMID: 41958549](https://pubmed.ncbi.nlm.nih.gov/41958549/). *Front Med (Lausanne)*. [Case Report / Case Series]
Bouma G (2025). [PMID: 41107015](https://pubmed.ncbi.nlm.nih.gov/41107015/). *Gastrointest Endosc Clin N Am*. [Review / Meta-Analysis]
Loghavi S (2025). [PMID: 39862928](https://pubmed.ncbi.nlm.nih.gov/39862928/). *Hum Pathol*. [Review / Meta-Analysis]
Dieckman T (2025). [PMID: 39802487](https://pubmed.ncbi.nlm.nih.gov/39802487/). *Gastro Hep Adv*. [Basic Science / Preclinical]
AI-curated news mentioning enteropathy-associated T-cell lymphoma
Updated Aug 11, 2026
Recent literature reviews and case studies highlight two instances of SMARCB1/INI1-deficient peripheral T-cell lymphoma, expanding the understanding of this rare entity. These findings contribute to the growing body of knowledge regarding this specific lymphoma subtype.
A new study explores the molecular clonal evolution of enteropathy-associated T-cell lymphoma, highlighting its clinical consequences. This research may provide insights into disease progression and treatment strategies.
A recent study published in PubMed examines real-world patient characteristics and treatment patterns for peripheral T-cell lymphoma in Japan. The research highlights healthcare resource utilization and associated costs, providing valuable insights for stakeholders in the rare disease space.