peripheral T-cell lymphoma, not otherwise specified
diseaseOn this page
Also known as PTCL
Summary
peripheral T-cell lymphoma, not otherwise specified (MONDO:0004964) is a cancer and 37 clinical trials. Top therapeutic interventions include cyclophosphamide anhydrous, pralatrexate, and romidepsin. A subtype of lymphoma — broader associated-gene and molecular evidence is on the parent page (see Disease family below).
At a glance
- Classification: Cancer
- Clinical trials: 37
Clinical features
No curated clinical features (Orphanet) for this disease.
Identifiers
Disease identifiers
| Field | Value |
|---|---|
| Canonical name | peripheral T-cell lymphoma, not otherwise specified |
| Mondo ID | MONDO:0004964 |
| EFO | EFO:0000211 |
| ICD-11 | 855224800 |
| NCIT | C4340 |
| GARD | 0027711 |
| Is cancer (heuristic) | yes |
Also known as: peripheral T-cell lymphoma, not otherwise specified · PTCL
Disease family
This is a subtype of lymphoma. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.
Classification path: disease › human disease › disease by etiologic mechanism › cancer or benign tumor › neoplastic disease or syndrome › neoplasm › hematopoietic and lymphoid system neoplasm › hematopoietic and lymphoid cell neoplasm › lymphoid neoplasm › lymphoma › peripheral T-cell lymphoma, not otherwise specified
Related subtypes (26): prostate lymphoma, nasal cavity lymphoma, bladder lymphoma, tracheal lymphoma, retroperitoneal lymphoma, ureteral lymphoma, ovarian lymphoma, B-cell lymphoma, unclassifiable, with features intermediate between diffuse large b-cell lymphoma and classical Hodgkin lymphoma, pediatric lymphoma, adult lymphoma, breast lymphoma, heart lymphoma, chest wall lymphoma, lung lymphoma, mediastinal malignant lymphoma, eye lymphoma, B-cell neoplasm, gastrointestinal lymphoma, Hodgkins lymphoma, composite lymphoma, AIDS-related primary central nervous system lymphoma, primary organ-specific lymphoma, non-Hodgkin lymphoma, extranodal nasal NK/T cell lymphoma, methotrexate-associated lymphoproliferative disorders, progressive transformation of germinal centers
Genetics & variants
GWAS landscape
No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.
Variant details and genetic-evidence tiers
No tiered GWAS variants or ClinVar records for this disease.
Genes & proteins
No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).
Function
No pathway enrichment — requires an associated-gene cohort.
Therapeutics
Drugs indicated for this disease
3 approved, 19 in late-stage (phase 3) trials. Disease-direct ChEMBL indications, not inferred from the associated-gene cohort below.
| Drug | Development status |
|---|---|
| Belinostat | Approved (phase 4) |
| Pralatrexate | Approved (phase 4) |
| Romidepsin | Approved (phase 4) |
| Alemtuzumab | Phase 3 (in late-stage trials) |
| Alisertib | Phase 3 (in late-stage trials) |
| Azacitidine | Phase 3 (in late-stage trials) |
| Cisplatin | Phase 3 (in late-stage trials) |
| Cladribine | Phase 3 (in late-stage trials) |
| Creatinolfosfate | Phase 3 (in late-stage trials) |
| Dacarbazine | Phase 3 (in late-stage trials) |
| Decitabine | Phase 3 (in late-stage trials) |
| Denileukin Diftitox | Phase 3 (in late-stage trials) |
| Doxorubicin | Phase 3 (in late-stage trials) |
| Epirubicin | Phase 3 (in late-stage trials) |
| Etoposide | Phase 3 (in late-stage trials) |
| Gemcitabine | Phase 3 (in late-stage trials) |
| Ifosfamide | Phase 3 (in late-stage trials) |
| Prednisone | Phase 3 (in late-stage trials) |
| Tucidinostat | Phase 3 (in late-stage trials) |
| Vasoactive Intestinal Peptide | Phase 3 (in late-stage trials) |
| Vinblastine | Phase 3 (in late-stage trials) |
| Vincristine | Phase 3 (in late-stage trials) |
Earlier-phase candidates (phase 2, investigational — efficacy not yet established): Bendamustine, Bleomycin, Bortezomib, Brentuximab Vedotin, Carmustine, Cyanocobalamin, Cytarabine, Dexamethasone, Duvelisib, Endostatin, N-Terminal-Mggshhhhh, Fenretinide, Filgrastim, Fludarabine, Fludarabine Phosphate, Folic Acid, Lenalidomide, Melphalan, Methotrexate, Methylprednisolone, Mycophenolate Mofetil, Nivolumab, Olaparib, Oxaliplatin, Pegfilgrastim, Pembrolizumab, Prednisolone, Selinexor, Sintilimab, Tacrolimus Anhydrous, Thiotepa, Tipifarnib, Vindesine.
Clinical trials & evidence
Clinical trials
Clinical trials: 37.
Phase distribution (across all retrieved trials)
| Phase | Trials |
|---|---|
| PHASE2 | 11 |
| PHASE1/PHASE2 | 9 |
| PHASE1 | 8 |
| Not specified | 7 |
| PHASE3 | 2 |
Top trials by phase / activity
| NCT | Phase | Status | Title |
|---|---|---|---|
| NCT06561048 | PHASE3 | RECRUITING | Soquelitinib vs Standard of Care in Participants With Relapsed/Refractory Peripheral T-cell Lymphoma Not Otherwise Specified, Follicular Helper T-cell Lymphomas, or Systemic Anaplastic Large-cell Lymphoma |
| NCT06724237 | PHASE3 | RECRUITING | Testing Whether High Dose Chemotherapy and Infusion of the Patients’ Own Stem Cells Improves Survival in Patients With Peripheral T-cell Lymphoma Who Achieved a Complete Response at the End of the Initial Chemotherapy |
| NCT03011814 | PHASE1/PHASE2 | ACTIVE_NOT_RECRUITING | Durvalumab With or Without Lenalidomide in Treating Patients With Relapsed or Refractory Cutaneous or Peripheral T Cell Lymphoma |
| NCT03113500 | PHASE2 | ACTIVE_NOT_RECRUITING | Brentuximab Vedotin and Combination Chemotherapy in Treating Patients With CD30-Positive Peripheral T-cell Lymphoma |
| NCT03278782 | PHASE1/PHASE2 | ACTIVE_NOT_RECRUITING | Study of Pembrolizumab (MK-3475) in Combination With Romidepsin |
| NCT03598998 | PHASE1/PHASE2 | ACTIVE_NOT_RECRUITING | Pembrolizumab and Pralatrexate in Treating Patients With Relapsed or Refractory Peripheral T-Cell Lymphomas |
| NCT04747236 | PHASE2 | RECRUITING | Randomized Phase IIB Trial of Oral Azacytidine Plus Romidepsin Versus Investigator’s Choice in PTCL |
| NCT06492629 | PHASE2 | ACTIVE_NOT_RECRUITING | Toripalimab Combined With Chidamide for the Treatment of Relapsed/Refractory Peripheral T-Cell Lymphoma |
| NCT06874946 | PHASE1/PHASE2 | RECRUITING | Nanobody-Based Anti-CD5 CAR-T for Relapsed/Refractory T-ALL/NHL |
| NCT07293403 | PHASE2 | NOT_YET_RECRUITING | Duvelisib Maintenance for the Treatment of Peripheral T-Cell Lymphomas |
| NCT07347288 | PHASE2 | NOT_YET_RECRUITING | SHR2554 as Maintenance Therapy in Patients With Peripheral T-cell Lymphoma |
| NCT07493330 | PHASE1/PHASE2 | NOT_YET_RECRUITING | Genotype-guided Targeted Agents Plus EZH2i for Primary Refractory PTCL |
| NCT02533700 | PHASE2 | UNKNOWN | CEOP/IVE/GDP Compared With CEOP as the First-line Therapy for Newly Diagnosed Adult Patients With PTCL |
| NCT02561273 | PHASE1/PHASE2 | COMPLETED | Combination Chemotherapy & Lenalidomide in Newly Diagnosed Stage II-IV Peripheral T-cell Non-Hodgkin’s Lymphoma |
| NCT03075553 | PHASE2 | TERMINATED | Nivolumab in Treating Patients With Relapsed or Refractory Peripheral T-cell Lymphoma |
| NCT03493451 | PHASE2 | COMPLETED | Study of BGB-A317 in Participants With Relapsed or Refractory Mature T- and NK-cell Neoplasms |
| NCT03590574 | PHASE1/PHASE2 | TERMINATED | Phase I/II Study Evaluating AUTO4 in Patients With T Cell Receptor Beta Constant (TRBC)1 Positive T Cell Lymphoma |
| NCT04074746 | PHASE1/PHASE2 | COMPLETED | Modified Immune Cells (AFM13-NK) and A Monoclonal Antibody (AFM13) in Treating Patients With Recurrent or Refractory CD30 Positive Hodgkin or Non-Hodgkin Lymphomas |
| NCT04705090 | PHASE2 | UNKNOWN | A Study of YY-20394 in Patients With Relapsed or Refractory Peripheral T/NK Cell Lymphoma |
| NCT04795869 | PHASE2 | WITHDRAWN | Brentuximab Vedotin and Pembrolizumab in Treating Patients With Recurrent Peripheral T-Cell Lymphoma |
| NCT04803201 | PHASE2 | SUSPENDED | Testing the Addition of Duvelisib or CC-486 to the Usual Treatment for Peripheral T-Cell Lymphoma |
| NCT05238064 | PHASE1/PHASE2 | UNKNOWN | Parsaclisib in Combination With CHOP in Participants With Previously Untreated PTCL |
| NCT03017820 | PHASE1 | RECRUITING | 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 |
| NCT03240211 | PHASE1 | ACTIVE_NOT_RECRUITING | Study of Pembrolizumab Combined With Decitabine and Pralatrexate in PTCL and CTCL |
| NCT06508463 | PHASE1 | RECRUITING | Intravenous Vesicular Stomatitis Virus in Patients With Peripheral T-cell Lymphoma |
| NCT06712810 | PHASE1 | RECRUITING | Q702 for the Treatment of Patients With Hematologic Malignancies |
| NCT07278856 | PHASE1 | NOT_YET_RECRUITING | Ruxolitinib in Combination With CHOP Chemotherapy for the Treatment of Untreated Nodal T-Follicular Helper Cell Lymphomas |
| NCT01590732 | PHASE1 | COMPLETED | Romidepsin, Ifosfamide, Carboplatin, and Etoposide in Treating Participants With Relapsed or Refractory Peripheral T-Cell Lymphoma |
| NCT02757248 | PHASE1 | WITHDRAWN | Ph1 Volasertib Plus Romidepsin in R/R PTCL and CTCL |
| NCT03900442 | PHASE1 | COMPLETED | Phase 1 Study of PTX-100 in Patients With Advanced Malignancies With PTCL Expansion Cohort |
| NCT01787409 | Not specified | ACTIVE_NOT_RECRUITING | Cholecalciferol in Improving Survival in Patients With Newly Diagnosed Cancer With Vitamin D Insufficiency |
| NCT05967949 | Not specified | RECRUITING | A Retrospective Clinical Study on the First-line Maintenance Treatment of PTCL With Chidamide. |
| NCT05978141 | Not specified | RECRUITING | A Registry for People With T-cell Lymphoma |
| NCT02652715 | Not specified | COMPLETED | Salvia Hispanica Seed in Reducing Risk of Disease Recurrence in Patients With Non-Hodgkin Lymphoma |
| NCT03040206 | Not specified | COMPLETED | Risk Stratification of Nodal PTCL |
| NCT03884205 | Not specified | UNKNOWN | GDPE/CEOPE Compared With CEOPE for Newly Diagnosed Patients With PTCL |
| NCT05821192 | Not specified | UNKNOWN | Chemotherapy Plus PD-1 Monoclonal Antibody in the Treatment of Refractory or Relapsed Peripheral T-cell Lymphoma. |
Drugs tested across these trials (top 30)
| Molecule | Max phase | Trials referencing |
|---|---|---|
| CYCLOPHOSPHAMIDE ANHYDROUS | 4 | 5 |
| PRALATREXATE | 4 | 4 |
| ROMIDEPSIN | 4 | 4 |
| BELINOSTAT | 4 | 2 |
| DUVELISIB | 4 | 2 |
| BRENTUXIMAB VEDOTIN | 4 | 1 |
| ETOPOSIDE PHOSPHATE | 4 | 1 |
| RUXOLITINIB | 4 | 1 |
| TISLELIZUMAB | 4 | 1 |
| PARSACLISIB | 3 | 1 |
| VOLASERTIB | 3 | 1 |
| ACIMTAMIG | 2 | 1 |
| GOLIDOCITINIB | 2 | 1 |
| SOQUELITINIB | 2 | 1 |
| ZEPRUMETOSTAT | 2 | 1 |
| GGTI-2418 | 1 | 1 |
| CHEMBL4066465 | 0 | 4 |
| CHEMBL3805348 | 0 | 2 |
| CHEMBL4538684 | 0 | 2 |
| CHEMBL4087968 | 0 | 1 |
| CHEMBL4284151 | 0 | 1 |