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Teaching pack: Peripheral T-cell lymphomas (including cutaneous T-cell lymphoma)

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  1. Teaching pack · Cancer · haematologic

    Peripheral T-cell lymphomas (including cutaneous T-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.

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  2. What it is

    In two paragraphs

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 Relapsed disease is treated with pralatrexate (2009), the HDAC inhibitors romidepsin (2009, US PTCL indication withdrawn 2021) and belinostat (2014), brentuximab, or allogeneic transplant.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

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  3. Standard of care

    What is given today, by setting

    SettingApproachGuideline
    First line, CD30+ PTCL / ALCLBrentuximab vedotin + CHP ×6 (ECHELON-2, OS benefit) ± consolidative autologous transplant.NCCN Category 1 (ALCL), ESMO-MCBS 4
    First line, other nodal PTCLCHOP or CHOEP (≤60 years) ×6, autologous transplant consolidation in responders; clinical trial preferred.NCCN Category 2A
    Relapsed/refractory PTCLPralatrexate, belinostat, romidepsin (ex-US), brentuximab (CD30+), gemcitabine-based regimens, allogeneic HSCT for fit responders.NCCN Category 2A
    Mycosis fungoides / SézarySkin-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)
    Extranodal NK/T-cellAsparaginase-based chemotherapy (P-GemOx, SMILE) with involved-site radiotherapy for localised disease; PD-1 inhibitors at relapse.not mapped
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  4. State of the art

    Where the field stands

    • 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.
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  5. History

    How we got here

    1. 1975Mycosis fungoides staging (TNMB)
    2. 1994ALK fusion in anaplastic large-cell lymphoma
    3. 2009Pralatrexate and romidepsin approved
    4. 2011Brentuximab vedotin approved for ALCL
    5. 2014Belinostat approved
    6. 2017ALCANZA: brentuximab in CD30+ CTCL
    7. 2018ECHELON-2 and mogamulizumab
    8. 2021Romidepsin US PTCL indication withdrawn
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  6. Pipeline

    What is coming

    • Brentuximab vedotin (product)
    • Azacitidine (product)
    • Allogeneic stem cell transplantation (technology)
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  7. Open problems

    What nobody has solved

    • 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.
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  8. Quiz

    Check understanding

    1. What is the standard treatment for stage II-III triple-negative breast cancer today?
      Answer
      Neoadjuvant pembrolizumab with carboplatin/paclitaxel then anthracycline chemotherapy, surgery, and adjuvant pembrolizumab (KEYNOTE-522); adjuvant olaparib for germline BRCA carriers with residual disease (OlympiA); capecitabine for residual disease without BRCA.
    2. Which biomarkers must be tested at diagnosis of advanced non-small-cell lung cancer?
      Answer
      EGFR, ALK, ROS1, BRAF V600E, MET exon 14/amplification, RET, NTRK, KRAS G12C, HER2 mutations, and PD-L1 TPS.
    3. What is the longest progression-free survival ever reported for a targeted pill in metastatic lung cancer?
      Answer
      Lorlatinib in ALK-positive NSCLC (CROWN): five-year PFS about 60% versus 8% for crizotinib.
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  9. Sources

    Read the primary sources

    • 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
    • Wikipedia: https://en.wikipedia.org/wiki/Peripheral_T-cell_lymphoma
    • Guideline: https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1483
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