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Aggressive nodal or extranodal mature (peripheral) T-cell lymphomas that do not belong to the better defined entities of the remainder of mature T-cell lymphomas. This category includes the following variants: lymphoepithelioid cell variant (Lennert's lymphoma), follicular variant, and T-zone variant.
Biomarker and diagnostic research for peripheral T-cell lymphoma, not otherwise specified has been reported in the published literature.
No approved treatments are currently available for peripheral T-cell lymphoma, not otherwise specified. An additional 6 compounds hold orphan drug designation.
While no drugs are FDA-approved specifically for peripheral T-cell lymphoma, not otherwise specified, 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 peripheral T-cell lymphoma, not otherwise specified. Orphan designation reflects regulatory interest and does not indicate approval for treatment.
Brand Name | Generic Name | Sponsor |
|---|
64 clinical trials registered, 38 recruiting. Interventions under study include drug therapy, other interventions, biologic therapy, and procedural interventions. Pipeline includes 6 PHASE3, 17 PHASE2, 26 PHASE1. Research is sponsored by a mix of industry and academic institutions.
NCT ID | Title | Phase | Sponsor | Status |
|---|---|---|---|---|
[NCT01137825](https://clinicaltrials.gov/study/NCT01137825) |
Data assembled from 4 of 12 sources · Last updated Oct 3, 2026, 6:00 AM UTC
Designated
Exclusivity End |
|---|
Designation Status |
|---|
4-[[(1S)-1-(4, 8-Dichloro-1-oxo-2-phenyl-3-isoquinolyl) ethyl] amino]-8H- pyrido[2,3-d] pyrimidin-5-one] | 4-[[(1S)-1-(4, 8-Dichloro-1-oxo-2-phenyl-3-isoquinolyl) ethyl] amino]-8H- pyrido[2,3-d] pyrimidin-5-one] | Boryung Co., LTD | 2022 | — | Designated |
Valemetostat tosylate | Valemetostat tosylate | Daiichi Sankyo, Inc. | 2021 | — | Designated |
cerdulatinib | cerdulatinib | Portola Pharmaceuticals, Inc. | 2018 | — | Designated |
devimistat | devimistat | Cornerstone Pharmaceuticals, Inc. | 2018 | — | Withdrawn |
denileukin diftitox | denileukin diftitox | Citius Pharmaceuticals, Inc. | 2011 | — | Designated |
Beleodaq | Belinostat | Acrotech Biopharma LLC | 2009 | 2021 | Designated (drug approved for other indication) |
Gene therapy approaches for peripheral T-cell lymphoma, not otherwise specified have been reported in the published literature.
64 trials found
Registry of Older Patients With Cancer |
— |
UNC Lineberger Comprehensive Cancer Center |
RECRUITING |
[NCT04234048](https://clinicaltrials.gov/study/NCT04234048) | Phase 1 Trial of ST-001 nanoFenretinide in Relapsed/Refractory T-cell Non-Hodgkin Lymphoma | PHASE1 | SciTech Development, Inc. | RECRUITING |
[NCT06421948](https://clinicaltrials.gov/study/NCT06421948) | Linperlisib Combined With Chidamide in Patients With PTCL | PHASE1 | Yanyan Liu | RECRUITING |
[NCT04512534](https://clinicaltrials.gov/study/NCT04512534) | Sintilimab Combined With Chidamide in Treating Peripheral T Cell Lymphoma | PHASE2 | Fudan University | RECRUITING |
[NCT03017820](https://clinicaltrials.gov/study/NCT03017820) | A Vaccine (VSV-hIFNβ-NIS) With or Without Cyclophosphamide and Combinations of Ipilimumab, Nivolumab, and Cemiplimab in Treating Relapsed or Refractory Multiple Myeloma, Acute Myeloid Leukemia or Lymphoma | PHASE1 | Mayo Clinic | RECRUITING |
96 publications have been identified in PubMed for peripheral T-cell lymphoma, not otherwise specified. Research spans Case Report / Case Series (42%), Review / Meta-Analysis (17%), and Epidemiology / Natural History (17%).
Research Type | Count | % of Total |
|---|---|---|
Patient case studies | 40 | 42% |
Research summaries | 16 | 17% |
Disease patterns and progression | 16 | 17% |
Testing and diagnosis research | 9 | 9% |
Clinical study results | 7 | 7% |
Laboratory research | 4 | 4% |
Other research | 2 | 2% |
New treatment approaches | 2 | 2% |
Hoogstra DJ (2026). [PMID: 42237783](https://pubmed.ncbi.nlm.nih.gov/42237783/). *Haematologica*. [Review / Meta-Analysis]
Zhu F (2026). [PMID: 41585010](https://pubmed.ncbi.nlm.nih.gov/41585010/). *Blood Sci*. [Basic Science / Preclinical]
Vinodhini M (2026). [PMID: 42040712](https://pubmed.ncbi.nlm.nih.gov/42040712/). *Indian J Hematol Blood Transfus*. [Epidemiology / Natural History]
Kurashige M (2026). [PMID: 41565277](https://pubmed.ncbi.nlm.nih.gov/41565277/). *J Clin Exp Hematop*. [Case Report / Case Series]
Geller S (2026). [PMID: 41296329](https://pubmed.ncbi.nlm.nih.gov/41296329/). *JAMA Dermatol*. [Case Report / Case Series]
Bledsoe JR (2026). [PMID: 42162914](https://pubmed.ncbi.nlm.nih.gov/42162914/). *Mod Pathol*. [Basic Science / Preclinical]
Awamleh García P (2026). [PMID: 42130928](https://pubmed.ncbi.nlm.nih.gov/42130928/). *Eur J Case Rep Intern Med*. [Case Report / Case Series]
Wei C (2026). [PMID: 41773975](https://pubmed.ncbi.nlm.nih.gov/41773975/). *Leuk Lymphoma*. [Clinical Trial Publication]
Zhang L (2026). [PMID: 41795995](https://pubmed.ncbi.nlm.nih.gov/41795995/). *Zhonghua Bing Li Xue Za Zhi*. [Case Report / Case Series]
Wang WJ (2026). [PMID: 41721448](https://pubmed.ncbi.nlm.nih.gov/41721448/). *Cytometry B Clin Cytom*. [Clinical Trial Publication]