# Peripheral T-cell lymphomas (including cutaneous T-cell lymphoma)

Source: https://onco.cc/cancers/peripheral-t-cell-lymphoma/  
OnCo record `peripheral-t-cell-lymphoma` (Cancer). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

Peripheral T-cell lymphomas are lymphomas of T cells rather than B cells. They are rarer, more varied and, apart from a few subtypes, harder to treat than B-cell lymphomas; several new drugs help only defined subtypes.

## Summary

PTCLs are a heterogeneous group: PTCL-NOS, nodal T-follicular-helper lymphomas (angioimmunoblastic), ALK-positive and ALK-negative anaplastic large-cell lymphoma (ALCL), adult T-cell leukaemia/lymphoma (HTLV-1), extranodal NK/T-cell lymphoma (EBV), enteropathy-associated and hepatosplenic T-cell lymphoma, and the cutaneous T-cell lymphomas (mycosis fungoides, Sézary syndrome). Except ALK-positive ALCL, five-year survival with CHOP is 30-40%.

CHOP or CHOEP remains the backbone; brentuximab vedotin-CHP replaced CHOP for CD30-positive PTCL after ECHELON-2 (2018) and is the standard for ALCL. Autologous transplant consolidation in first remission is common practice without randomised proof. Relapsed disease is treated with pralatrexate (2009), the HDAC inhibitors romidepsin (2009, US PTCL indication withdrawn 2021) and belinostat (2014), brentuximab, or allogeneic transplant. NK/T-cell lymphoma uses asparaginase-based regimens (SMILE, P-GemOx) and radiotherapy; PD-1 blockade is active. Cutaneous T-cell lymphoma is managed by skin-directed therapy, then mogamulizumab (anti-CCR4, MAVORIC 2018), brentuximab (ALCANZA), bexarotene, extracorporeal photopheresis, and allogeneic transplant.

Open: TFH-lymphoma-directed epigenetic combinations (azacitidine-CHOP, ORACLE), CD30/CD7/CD5 CAR-T with fratricide engineering, and JAK/STAT inhibitors.

## Fields

- Kind: Cancer
- Last checked: 2026-09-08
- Also known as: PTCL; CTCL; Mycosis fungoides; Anaplastic large-cell lymphoma
- Tags: gap-fill; haematologic
- Group: haematologic
- Burden: Peripheral T-cell lymphomas make up about 10-15% of non-Hodgkin lymphomas in the West, more in Asia; there are over 30 WHO subtypes, most individually rare.
- Subtypes: PTCL, not otherwise specified; Nodal TFH lymphoma (angioimmunoblastic type); ALK-positive ALCL; ALK-negative ALCL (DUSP22, TP63 subsets); Adult T-cell leukaemia/lymphoma (HTLV-1); Extranodal NK/T-cell lymphoma (EBV); Mycosis fungoides / Sézary syndrome (CTCL); Enteropathy-associated and hepatosplenic T-cell lymphoma
- Biomarkers: CD30 expression (brentuximab); ALK rearrangement; TFH markers (PD-1, CXCL13, ICOS) and RHOA G17V / TET2 / IDH2 R172; EBV DNA (NK/T-cell); HTLV-1 serology; CCR4 (mogamulizumab); TCR clonality

## Sections of this record

The page is a hub with ten sections in reading order; large sections have their own page. The same plan as JSON: https://onco.cc/api/v1/cancers/peripheral-t-cell-lymphoma/sections.json

- Overview (on the hub): The TL;DR, the family this cancer belongs to, the organ, who gets it and what the state of the art is. https://onco.cc/cancers/peripheral-t-cell-lymphoma/#overview [3 subtypes, 4 state-of-the-art points]
- What it is (on the hub): Anatomy, the subtypes and how they differ, how it is staged, and where advanced disease spreads. https://onco.cc/cancers/peripheral-t-cell-lymphoma/#what-it-is [11 subtypes]
- Finding it (on the hub): How it shows itself, how it is confirmed, what screening exists, and the biomarkers clinicians test for. https://onco.cc/cancers/peripheral-t-cell-lymphoma/#finding-it [7 biomarkers, 3 prevalence rows]
- Treating it (on the hub): The standard of care by setting, the medicines, surgery and radiotherapy named in it, and the regimens behind them. https://onco.cc/cancers/peripheral-t-cell-lymphoma/#treating-it [5 settings, 1 regimen, 5 decisions with options]
- Evidence (on the hub): Trials recruiting now, the landmark trials, the key papers and what they mean, the latest literature, and the milestones year by year. https://onco.cc/cancers/peripheral-t-cell-lymphoma/#evidence [31 trials, 1 key paper, 8 milestones]
- The science (on the hub): The molecular landscape: the targets and how often each appears, the pathways, the mechanics stages and the preclinical models. https://onco.cc/cancers/peripheral-t-cell-lymphoma/#science [16 targets]
- Where you are (own page): Cases by country, the UK and NHS pathway and other country lenses, and the expert centres with trials on record. https://onco.cc/cancers/peripheral-t-cell-lymphoma/where-you-are/
- Living with it (on the hub): The decisions you may face, the aids that walk through them, the warnings on record, the first sixty days and the questions to ask. https://onco.cc/cancers/peripheral-t-cell-lymphoma/#living-with-it [19 questions, 6 red cards]
- What is coming (own page): Everything in development, the open problems and what is being done about them, the roadmaps, and what changed on this record. https://onco.cc/cancers/peripheral-t-cell-lymphoma/coming/ [20 medicines, 31 trials, 4 open problems]
- Data (own page): Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked. https://onco.cc/cancers/peripheral-t-cell-lymphoma/data/ [111 connected records]

## Standard of care

- First line, CD30+ PTCL / ALCL: Brentuximab vedotin + CHP ×6 (ECHELON-2, OS benefit) ± consolidative autologous transplant. ([Brentuximab vedotin](https://onco.cc/drugs/brentuximab-vedotin/), [Autologous stem cell transplant (high-dose therapy)](https://onco.cc/technologies/autologous-stem-cell-transplant/))
- First line, other nodal PTCL: CHOP or CHOEP (≤60 years) ×6, autologous transplant consolidation in responders; clinical trial preferred. ([Doxorubicin](https://onco.cc/drugs/doxorubicin/), [Cyclophosphamide](https://onco.cc/drugs/cyclophosphamide/), [Vincristine](https://onco.cc/drugs/vincristine/), [Autologous stem cell transplant (high-dose therapy)](https://onco.cc/technologies/autologous-stem-cell-transplant/))
- Relapsed/refractory PTCL: Pralatrexate, belinostat, romidepsin (ex-US), brentuximab (CD30+), gemcitabine-based regimens, allogeneic HSCT for fit responders. ([Pralatrexate](https://onco.cc/drugs/pralatrexate/), [Belinostat](https://onco.cc/drugs/belinostat/), [Romidepsin](https://onco.cc/drugs/romidepsin/), [Brentuximab vedotin](https://onco.cc/drugs/brentuximab-vedotin/), [Allogeneic stem cell transplantation](https://onco.cc/technologies/allogeneic-hsct/))
- Mycosis fungoides / Sézary: Skin-directed therapy (topical steroids, phototherapy, radiotherapy, total-skin electron beam), then mogamulizumab (MAVORIC), brentuximab (ALCANZA, CD30+), bexarotene, interferon, photopheresis; allogeneic HSCT for advanced disease. ([Mogamulizumab](https://onco.cc/drugs/mogamulizumab/), [Brentuximab vedotin](https://onco.cc/drugs/brentuximab-vedotin/), [Allogeneic stem cell transplantation](https://onco.cc/technologies/allogeneic-hsct/))
- Extranodal NK/T-cell: Asparaginase-based chemotherapy (P-GemOx, SMILE) with involved-site radiotherapy for localised disease; PD-1 inhibitors at relapse. ([Pembrolizumab](https://onco.cc/drugs/pembrolizumab/), [IMRT / IGRT (modern external beam)](https://onco.cc/technologies/imrt-igrt/))

## State of the art

- Brentuximab-CHP is the only regimen ever to improve survival over CHOP in PTCL, and only for CD30-positive disease.
- Molecular subtyping (TFH, DUSP22, TP63) now guides prognosis and trial design, though not yet routine therapy.
- Mogamulizumab and brentuximab give durable disease control in advanced cutaneous T-cell lymphoma.
- Epigenetic therapy (azacitidine, HDAC inhibitors) is most active in TFH lymphomas with TET2/DNMT3A mutations.

## Open problems

- Five-year survival under 40% for PTCL-NOS with no new first-line regimen for non-CD30 disease.
- Randomised evidence for transplant consolidation is lacking.
- T-cell CAR-T faces fratricide and T-cell aplasia.
- Most subtypes too rare for conventional phase 3 trials.

## Sources

- Wikipedia: https://en.wikipedia.org/wiki/Peripheral_T-cell_lymphoma
- NCCN Guidelines: T-Cell Lymphomas: https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1483
- ECHELON-2 (Lancet 2019): https://doi.org/10.1016/S0140-6736(18)32984-2
- NCI PDQ: mycosis fungoides: https://www.cancer.gov/types/lymphoma/patient/mycosis-fungoides-treatment-pdq

## Connected records

- cancers: [Cutaneous T-cell lymphoma (mycosis fungoides and Sezary syndrome)](https://onco.cc/cancers/cutaneous-t-cell-lymphoma/), [Diffuse large B-cell lymphoma](https://onco.cc/cancers/dlbcl/), [Follicular lymphoma](https://onco.cc/cancers/follicular-lymphoma/), [Hepatosplenic T-cell lymphoma](https://onco.cc/cancers/hepatosplenic-t-cell-lymphoma/), [Mantle cell lymphoma](https://onco.cc/cancers/mantle-cell-lymphoma/), [Nodal T-follicular helper cell lymphoma, angioimmunoblastic type (angioimmunoblastic T-cell lymphoma)](https://onco.cc/cancers/angioimmunoblastic-t-cell-lymphoma/), [Non-Hodgkin lymphoma (all types)](https://onco.cc/cancers/non-hodgkin-lymphoma/), [Sezary syndrome](https://onco.cc/cancers/sezary-syndrome/)
- technologies: [Allogeneic stem cell transplantation](https://onco.cc/technologies/allogeneic-hsct/), [Antibody-drug conjugate (ADC)](https://onco.cc/technologies/adc/), [Autologous stem cell transplant (high-dose therapy)](https://onco.cc/technologies/autologous-stem-cell-transplant/), [CAR-T cell therapy](https://onco.cc/technologies/car-t/), [Epigenetic drugs (HDAC, DNMT, EZH2, IDH, menin, BET)](https://onco.cc/technologies/epigenetic-drugs/), [IMRT / IGRT (modern external beam)](https://onco.cc/technologies/imrt-igrt/), [Monoclonal antibodies](https://onco.cc/technologies/monoclonal-antibody/), [Total skin electron beam therapy](https://onco.cc/technologies/total-skin-electron-therapy/)
- targets: [ALK](https://onco.cc/targets/alk/), [CCR4](https://onco.cc/targets/ccr4/), [CD25 (IL-2 receptor alpha)](https://onco.cc/targets/cd25/), [CD30](https://onco.cc/targets/cd30/), [CD52](https://onco.cc/targets/cd52/), [CD7](https://onco.cc/targets/cd7/), [CD80 (B7-1)](https://onco.cc/targets/cd80/), [Histone deacetylases (HDAC)](https://onco.cc/targets/hdac/), [JAK1](https://onco.cc/targets/jak1/), [PD-1](https://onco.cc/targets/pd1/), [TET2](https://onco.cc/targets/tet2/)
- drugs: [Asparaginase (pegaspargase, calaspargase pegol, Erwinia asparaginase)](https://onco.cc/drugs/asparaginase/), [Azacitidine](https://onco.cc/drugs/azacitidine/), [AZD3470](https://onco.cc/drugs/azd3470/), [Belinostat](https://onco.cc/drugs/belinostat/), [Brentuximab vedotin](https://onco.cc/drugs/brentuximab-vedotin/), [Crizotinib](https://onco.cc/drugs/crizotinib/), [Cyclophosphamide](https://onco.cc/drugs/cyclophosphamide/), [Doxorubicin](https://onco.cc/drugs/doxorubicin/), [DR-01](https://onco.cc/drugs/dr-01/), [Duvelisib](https://onco.cc/drugs/duvelisib/), [Golidocitinib](https://onco.cc/drugs/golidocitinib/), [Methoxsalen (extracorporeal photopheresis)](https://onco.cc/drugs/methoxsalen-ecp/), [Mogamulizumab](https://onco.cc/drugs/mogamulizumab/), [Pembrolizumab](https://onco.cc/drugs/pembrolizumab/), [Pralatrexate](https://onco.cc/drugs/pralatrexate/), [Romidepsin](https://onco.cc/drugs/romidepsin/), [Soquelitinib](https://onco.cc/drugs/soquelitinib/), [Sugemalimab](https://onco.cc/drugs/sugemalimab/), [Tucidinostat (chidamide)](https://onco.cc/drugs/tucidinostat/), [Vincristine](https://onco.cc/drugs/vincristine/)
- companies: [Acrotech Biopharma](https://onco.cc/companies/acrotech-biopharma/), [BioInvent International](https://onco.cc/companies/bioinvent-international/), [Bristol Myers Squibb](https://onco.cc/companies/bms/), [Evopoint Biosciences](https://onco.cc/companies/evopoint-biosciences/), [Haihe Biopharma](https://onco.cc/companies/haihe-biopharma/), [HUYABIO International](https://onco.cc/companies/huyabio/), [ImmuneOncia Therapeutics](https://onco.cc/companies/immuneoncia-therapeutics/), [Mundipharma](https://onco.cc/companies/mundipharma/), [Pfizer (incl. Seagen)](https://onco.cc/companies/pfizer/), [Takeda](https://onco.cc/companies/takeda/)
- terms: [Histologic transformation](https://onco.cc/terms/histologic-transformation/), [Lugano classification / Ann Arbor staging](https://onco.cc/terms/lugano-classification/)
- trials: [A Phase 1/2 Study of STP938 (Dencatistat) for Adult Subjects With Relapsed/Refractory B-Cell and T-Cell Lymphomas](https://onco.cc/trials/nct05463263/), [A Phase 3 Multinational Study of Golidocitinib Versus Investigator's Choice in r/r PTCL (JACKPOT19)](https://onco.cc/trials/nct07234162/), [A Phase IIa Clinical Study of Purinostat Mesylate for Injection in Patients With Peripheral T-Cell Lymphoma and Cutaneous T-Cell Lymphoma](https://onco.cc/trials/nct06485219/), [A Randomized, Double-blind, Multicenter Phase III Study of XNW5004 Tablets in Patients With Relapsed or Refractory Peripheral T-cell Lymphoma](https://onco.cc/trials/nct06776952/), [A Study of Brentuximab Vedotin in Combination With Cyclophosphamide, Doxorubicin (Hydroxydaunorubicin), Prednisone (CHP) in Chinese Participants With CD30-Positive (CD30+) Peripheral T-Cell Lymphomas (PTCL)](https://onco.cc/trials/nct05673785/), [A Study of DR-01 in Subjects With Large Granular Lymphocytic Leukemia or Cytotoxic Lymphomas](https://onco.cc/trials/nct05475925/), [A Study of GFH009 Monotherapy in Patients with Relapsed or Refractory Peripheral T-cell Lymphoma (PTCL)](https://onco.cc/trials/nct05934513/), [A Study of GNC-038 Injection in Patients With Relapsed or Refractory NK/ T-cell Lymphoma, AITL, and Other NHL](https://onco.cc/trials/nct05627856/), [A Study of IMC-001 in Subjects With Relapsed or Refractory Extranodal NK/T Cell Lymphoma, Nasal Type](https://onco.cc/trials/nct04414163/), [A Study to Assess the Safety, Tolerability, Efficacy, and Drug Levels of BMS-986369 (Golcadomide) in Participants With Relapsed or Refractory T-cell Lymphomas in Japan (GOLSEEK-3)](https://onco.cc/trials/nct06035497/), [Adaptive Dual-Target CAR-T Cells for Relapsed or Refractory Hematologic Malignancies](https://onco.cc/trials/nct07523555/), [ALCANZA](https://onco.cc/trials/alcanza/), [Assessing Treatment of T-Cell Lymphoma With GW5282 in Combination With Golidocitinib (BEI-DOU3)](https://onco.cc/trials/nct07496229/), [Chidamide in Patients With Relapsed or Refractory Peripheral T-cell Lymphoma (R/R PTCL)](https://onco.cc/trials/nct05833724/), [Clinical Study of Mitoxantrone Hydrochloride Liposome Injection vs. Chidamide in Patients With Relapsed/Refractory PTCL](https://onco.cc/trials/nct04668690/), [Clinical Study of XNW5004 Combined With CHOP/CHOEP in the Treatment of Untreated Peripheral T-Cell Lymphoma](https://onco.cc/trials/nct07730515/), [Confirmatory Study of Topical HyBryte™ vs. Placebo for the Treatment of CTCL](https://onco.cc/trials/nct06470451/), [ECHELON-2](https://onco.cc/trials/echelon-2/), [Evaluate the Safety and Clinical Activity of HH2853](https://onco.cc/trials/nct04390737/), [Extracorporeal Photopheresis and Mogamulizumab for the Treatment of Erythrodermic Cutaneous T Cell Lymphoma](https://onco.cc/trials/nct04930653/), [HyBryte (Synthetic Hypericin) Versus Valchlor (Mechlorethamine) in the Treatment of CTCL](https://onco.cc/trials/nct06149247/), [MAVORIC](https://onco.cc/trials/mavoric/), [MB-105 in Patients With CD5 Positive T-cell Lymphoma](https://onco.cc/trials/nct06534060/), [PUVA Maintenance Therapy in Mycosis Fungoides](https://onco.cc/trials/nct01686594/), [Randomized Phase IIB Trial of Oral Azacytidine Plus Romidepsin Versus Investigator's Choice in PTCL](https://onco.cc/trials/nct04747236/), [Resminostat for Maintenance Treatment of Patients With Advanced Stage Mycosis Fungoides (MF) or Sézary Syndrome (SS)](https://onco.cc/trials/nct02953301/), [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](https://onco.cc/trials/nct06561048/), [Study to Evaluate Efficacy and Safety of HH2853 in Relapsed/Refractory Peripheral T-cell Lymphoma](https://onco.cc/trials/nct06909877/), [To Evaluate Efficacy of Belinostat or Pralatrexate in Combination Against CHOP Alone in PTCL](https://onco.cc/trials/nct06072131/), [Tucidinostat in Combination With CHOP in Newly Diagnosed Peripheral T-Cell Lymphoma With Follicular Helper of T Cell Phenotype](https://onco.cc/trials/nct06947967/), [Valemetostat Tosylate (DS-3201b), an Enhancer of Zeste Homolog (EZH) 1/2 Dual Inhibitor, for Relapsed/Refractory Peripheral T-Cell Lymphoma (VALENTINE-PTCL01)](https://onco.cc/trials/nct04703192/)
- key papers: [Brentuximab vedotin with chemotherapy for CD30-positive peripheral T-cell lymphoma (ECHELON-2): a global, double-blind, randomised, phase 3 trial](https://onco.cc/key-papers/paper-horwitz-lancet/)
- people: [H. Miles Prince](https://onco.cc/people/miles-prince/), [Lim Soon Thye](https://onco.cc/people/lim-soon-thye/), [Steven M. Horwitz](https://onco.cc/people/steven-horwitz/), [Youn H. Kim](https://onco.cc/people/youn-kim/)
- collections: [Lymphoma Research Foundation (LRF)](https://onco.cc/collections/lymphoma-research-foundation/)
- biomarkers: [CD30 expression (CD30-positive)](https://onco.cc/biomarkers/cd30-expression/)

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JSON: https://onco.cc/api/v1/entities/peripheral-t-cell-lymphoma.json