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

aka PTCL, CTCL, Mycosis fungoides, Anaplastic large-cell lymphoma

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.

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 Relapsed disease is treated with pralatrexate (2009), the HDAC inhibitors romidepsin (2009, US PTCL indication withdrawn 2021) and belinostat (2014), brentuximab, or allogeneic transplant.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.

State of the art today

  • 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.
Show survival figures (2)

Averages across everyone diagnosed, often years ago. Your stage, subtype, age, fitness and the treatment you receive matter more than the average, and the numbers are improving quickly.

  • 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.
Who it affects

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.

Where the cases are

Cases by country · GLOBOCAN 2022
All countries →

No country-level case numbers. This cancer is not mapped to a GLOBOCAN site.

Standard of care

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First line, CD30+ PTCL / ALCL

Brentuximab vedotin + CHP ×6 (ECHELON-2, OS benefit) ± consolidative autologous transplant.

NCCN · Category 1 (ALCL)ESMO-MCBS · 4
Brentuximab vedotinAutologous stem cell transplant (high-dose therapy)
First line, other nodal PTCL

CHOP or CHOEP (≤60 years) ×6, autologous transplant consolidation in responders; clinical trial preferred.

NCCN · Category 2A
DoxorubicinCyclophosphamideVincristineAutologous stem cell transplant (high-dose therapy)
Relapsed/refractory PTCL

Pralatrexate, belinostat, romidepsin (ex-US), brentuximab (CD30+), gemcitabine-based regimens, allogeneic HSCT for fit responders.

NCCN · Category 2A
PralatrexateBelinostatRomidepsinBrentuximab vedotinAllogeneic stem cell transplantation
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.

NCCN · Category 1 (mogamulizumab, brentuximab)
MogamulizumabBrentuximab vedotinAllogeneic stem cell transplantation
Extranodal NK/T-cell

Asparaginase-based chemotherapy (P-GemOx, SMILE) with involved-site radiotherapy for localised disease; PD-1 inhibitors at relapse.

PembrolizumabIMRT / IGRT (modern external beam)

Subtypes & biomarkers

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Subtypes
Biomarkers clinicians test

Target prevalence in this cancer

History

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  1. 1975Mycosis fungoides staging (TNMB)
  2. 1994ALK fusion in anaplastic large-cell lymphoma

    NPM-ALK identified by Morris et al.; ALK-positive ALCL becomes the good-prognosis PTCL.

    ALK
  3. 2009Pralatrexate and romidepsin approved

    First drugs specifically for relapsed PTCL.

    PralatrexateRomidepsin
  4. 2011Brentuximab vedotin approved for ALCL
    Brentuximab vedotin
  5. 2014Belinostat approved
    Belinostat
  6. 2017ALCANZA: brentuximab in CD30+ CTCL
    Brentuximab vedotin
  7. 2018ECHELON-2 and mogamulizumab

    Brentuximab-CHP improves OS over CHOP; mogamulizumab approved for MF/Sézary (MAVORIC).

    Brentuximab vedotinMogamulizumab
  8. 2021Romidepsin US PTCL indication withdrawn

    Confirmatory Ro-CHOP trial negative.

    Romidepsin

Pipeline

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ApprovedADC
Brentuximab vedotin · Adcetris
ApprovedSmall-molecule hypomethylating agent (cytotoxic)
Azacitidine · Vidaza / Onureg (oral)
Allogeneic stem cell transplantation

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.

Trials

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Recruiting now (live from ClinicalTrials.gov)

Recruiting trials near you · live from ClinicalTrials.gov
Peripheral T-cell lymphomas (including cutaneous T-cell lymphoma)
condition: Peripheral T-cell lymphomas
Open on ClinicalTrials.gov →

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Expert centres

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Where the expertise is

Centres linked to this cancer in OnCo

Seeking a second opinion: ask your oncologist for a referral to a high-volume centre; most accept records and pathology by mail or telehealth. In the US, use the NCI's Find a Cancer Center tool or the nonprofit Cancer Commons, which navigates options for advanced cancers at no cost.

Questions to ask

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Bring to your appointment

Questions to ask your oncologist about Peripheral T-cell lymphomas

Generated from this cancer's standard of care, biomarkers, and pipeline · 19 questions

Newly diagnosed

  1. What is my exact diagnosis, stage, and grade, and which tests established them?
    Why: Everything else follows from an accurate stage and subtype.
  2. Which biomarkers have been tested on my tumour (for example CD30 expression, ALK rearrangement, TFH markersand RHOA G17V / TET2 / IDH2 R172, EBV DNA, HTLV-1 serology), and what were the results?
    Why: These results decide eligibility for targeted therapy, immunotherapy, and trials.
  3. Which subtype is my cancer, and does that change the recommended treatment?
    Why: Recognised subtypes for this cancer include PTCL, not otherwise specified, Nodal TFH lymphoma, ALK-positive ALCL.
  4. Is germline (inherited) genetic testing recommended for me or my family?
    Why: Inherited variants can change treatment and matter for relatives.

First line, CD30+ PTCL / ALCL

  1. For my situation (first line, cd30+ ptcl / alcl), which of the standard options do you recommend and why?
    Why: Guideline options include: Brentuximab vedotin + CHP ×6 (ECHELON-2, OS benefit) ± consolidative autologous transplant.
  2. Am I a candidate for Brentuximab vedotin, and what side effects should I expect?
    Why: Knowing the expected toxicities helps you plan work, family, and supportive care.

First line, other nodal PTCL

  1. For my situation (first line, other nodal ptcl), which of the standard options do you recommend and why?
    Why: Guideline options include: CHOP or CHOEP (≤60 years) ×6, autologous transplant consolidation in responders; clinical trial preferred.
  2. Am I a candidate for Doxorubicin, Cyclophosphamide, Vincristine, and what side effects should I expect?
    Why: Knowing the expected toxicities helps you plan work, family, and supportive care.

Relapsed/refractory PTCL

  1. For my situation (relapsed/refractory ptcl), which of the standard options do you recommend and why?
    Why: Guideline options include: Pralatrexate, belinostat, romidepsin (ex-US), brentuximab (CD30+), gemcitabine-based regimens, allogeneic HSCT for fit responders.
  2. Am I a candidate for Pralatrexate, Belinostat, Romidepsin or related drugs, and what side effects should I expect?
    Why: Knowing the expected toxicities helps you plan work, family, and supportive care.

Mycosis fungoides / Sézary

  1. For my situation (mycosis fungoides / sézary), which of the standard options do you recommend and why?
    Why: Guideline options include: 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.
  2. Am I a candidate for Mogamulizumab, Brentuximab vedotin, and what side effects should I expect?
    Why: Knowing the expected toxicities helps you plan work, family, and supportive care.

Extranodal NK/T-cell

  1. For my situation (extranodal nk/t-cell), which of the standard options do you recommend and why?
    Why: Guideline options include: Asparaginase-based chemotherapy (P-GemOx, SMILE) with involved-site radiotherapy for localised disease; PD-1 inhibitors at relapse.
  2. Am I a candidate for Pembrolizumab, and what side effects should I expect?
    Why: Knowing the expected toxicities helps you plan work, family, and supportive care.

Any stage

  1. Are there clinical trials I could join, for example of Brentuximab vedotin, Azacitidine, Allogeneic stem cell transplantation?
    Why: Trials are how the next standard of care is set; asking early keeps options open.
  2. Would a second opinion at a high-volume centre change anything, and can you help arrange it?
    Why: Rare or high-stakes decisions benefit from a centre that treats many similar patients.
  3. What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?
    Why: Supportive care improves quality of life and helps patients complete treatment.
  4. I read that “Five-year survival under 40% for PTCL-NOS with no new first-line regimen for non-CD30 disease”. How does that affect my plan?
    Why: Open problems are where trials and second opinions matter most.
  5. I read that “Randomised evidence for transplant consolidation is lacking”. How does that affect my plan?
    Why: Open problems are where trials and second opinions matter most.

Print this page for your appointment (your browser's print command). These prompts are for discussion; your clinical team knows your case.

Everything relevant

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Direct links plus the targets, companies, and technologies of this cancer's products.

technologies

11
Allogeneic stem cell transplantationAntibody-drug conjugate (ADC)Autologous stem cell transplant (high-dose therapy)CAR-T cell therapyCardio-oncologyCytotoxic chemotherapyEpigenetic drugs (HDAC, DNMT, EZH2, IDH, menin, BET)Immune checkpoint inhibitorsIMRT / IGRT (modern external beam)Monoclonal antibodiesSmall-molecule kinase inhibitors

targets

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ALKCCR4CD30CD52CD7METPD-1PIK3CA / PI3K-alphaROS1

drugs

13
Not mapped hereEnzyme therapy
Asparaginase (pegaspargase, calaspargase pegol, Erwinia asparaginase) · Oncaspar / Asparlas / Rylaze / Erwinaze
ApprovedSmall-molecule hypomethylating agent (cytotoxic)
Azacitidine · Vidaza / Onureg (oral)
Not mapped herePan-HDAC inhibitor (hydroxamate)
Belinostat · Beleodaq
ApprovedADC
Brentuximab vedotin · Adcetris
Not mapped hereSmall-molecule ALK/ROS1/MET TKI (first generation)
Crizotinib · Xalkori
Not mapped hereAlkylating agent (oxazaphosphorine prodrug)
Cyclophosphamide · Cytoxan / Endoxan
ApprovedCytotoxic chemotherapy (anthracycline)
Doxorubicin · Adriamycin
Not mapped hereSmall-molecule PI3Kδ/γ inhibitor
Duvelisib · Copiktra
Not mapped hereDefucosylated anti-CCR4 monoclonal antibody
Mogamulizumab · Poteligeo
ApprovedMonoclonal antibody (anti-PD-1)
Pembrolizumab · Keytruda / Keytruda Qlex (SC)
Not mapped hereAntifolate (DHFR inhibitor)
Pralatrexate · Folotyn
Not mapped hereClass I HDAC inhibitor (cyclic peptide)
Romidepsin · Istodax
Not mapped hereVinca alkaloid (microtubule inhibitor)
Vincristine · Oncovin / Marqibo

companies

6
Bristol Myers SquibbJazz PharmaceuticalsMerck & Co. (MSD)Pfizer (incl. Seagen)ServierTakeda

terms

2
Histologic transformationLugano classification / Ann Arbor staging

collections

1
Lymphoma Research Foundation (LRF)

Latest papers

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Latest papers · live from Europe PMC
Open in Europe PMC

Query for this cancer: (TITLE:"Peripheral T-cell lymphomas" OR ABSTRACT:"Peripheral T-cell lymphomas" OR TITLE:"including cutaneous T-cell lymphoma" OR ABSTRACT:"including cutaneous T-cell lymphoma" OR TITLE:"PTCL" OR ABSTRACT:"PTCL" OR TITLE:"CTCL" OR ABSTRACT:"CTCL" OR TITLE:"Mycosis fungoides" OR ABSTRACT:"Mycosis fungoides" OR TITLE:"Anaplastic large-cell lymphoma" OR ABSTRACT:"Anaplastic large-cell lymphoma") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Peripheral T-cell lymphomas (including cutaneous T-cell lymphoma), not a curated reading list.

Connected

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Pages like this

not linked directly; found by shared links

technologies

7
Allogeneic stem cell transplantationAntibody-drug conjugate (ADC)Autologous stem cell transplant (high-dose therapy)CAR-T cell therapyEpigenetic drugs (HDAC, DNMT, EZH2, IDH, menin, BET)IMRT / IGRT (modern external beam)Monoclonal antibodies

targets

6
ALKCCR4CD30CD52CD7PD-1

drugs

13
Not mapped hereEnzyme therapy
Asparaginase (pegaspargase, calaspargase pegol, Erwinia asparaginase) · Oncaspar / Asparlas / Rylaze / Erwinaze
ApprovedSmall-molecule hypomethylating agent (cytotoxic)
Azacitidine · Vidaza / Onureg (oral)
Not mapped herePan-HDAC inhibitor (hydroxamate)
Belinostat · Beleodaq
ApprovedADC
Brentuximab vedotin · Adcetris
Not mapped hereSmall-molecule ALK/ROS1/MET TKI (first generation)
Crizotinib · Xalkori
Not mapped hereAlkylating agent (oxazaphosphorine prodrug)
Cyclophosphamide · Cytoxan / Endoxan
ApprovedCytotoxic chemotherapy (anthracycline)
Doxorubicin · Adriamycin
Not mapped hereSmall-molecule PI3Kδ/γ inhibitor
Duvelisib · Copiktra
Not mapped hereDefucosylated anti-CCR4 monoclonal antibody
Mogamulizumab · Poteligeo
ApprovedMonoclonal antibody (anti-PD-1)
Pembrolizumab · Keytruda / Keytruda Qlex (SC)
Not mapped hereAntifolate (DHFR inhibitor)
Pralatrexate · Folotyn
Not mapped hereClass I HDAC inhibitor (cyclic peptide)
Romidepsin · Istodax
Not mapped hereVinca alkaloid (microtubule inhibitor)
Vincristine · Oncovin / Marqibo

companies

3
Bristol Myers SquibbPfizer (incl. Seagen)Takeda

terms

2
Histologic transformationLugano classification / Ann Arbor staging

collections

1
Lymphoma Research Foundation (LRF)