The first 60 days: 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. Below, week by week, is what OnCo's record of Peripheral T-cell lymphomas (including cutaneous T-cell lymphoma) says about the first two months: the order is typical, the timing is yours to ask about. Sections appear only where the record has something to say. Orientation, not medical advice.
What happens now
Staging tests establish exactly what and where the cancer is. Everything else follows from the answers.
Ask which of these were tested and what the results were; see the report reader for what each value means.
These specialties appear in the standard of care for this cancer. In most centres they meet weekly as a tumour board to agree each plan; you can ask when yours was discussed and what was decided.
- Medical oncologistNamed in the standard of care for: First line, CD30+ PTCL / ALCL, First line, other nodal PTCL, Relapsed/refractory PTCL, Mycosis fungoides / Sézary and 1 more.
- Clinical oncologist (radiotherapy)Named in the standard of care for: Mycosis fungoides / Sézary, Extranodal NK/T-cell.
- Transplant and cell therapy teamNamed in the standard of care for: First line, CD30+ PTCL / ALCL, First line, other nodal PTCL, Relapsed/refractory PTCL, Mycosis fungoides / Sézary.
- Palliative and supportive care teamNamed in the standard of care for: First line, other nodal PTCL.
A second opinion from a centre that treats many similar cases is normal, not rude; the standard of care tells you what to compare it against.
Each row is a setting from the standard of care, in the order it usually arises. Not all will apply to you; your stage and biomarkers decide which do. The guideline grade, where recorded, says how strong the evidence is.
- 1.First line, CD30+ PTCL / ALCLNCCN category Category 1 (ALCL), ESMO-MCBS 4, NCCN Guidelines: T-Cell Lymphomas
Brentuximab vedotin + CHP ×6 (ECHELON-2, OS benefit) ± consolidative autologous transplant.
CHOP or CHOEP (≤60 years) ×6, autologous transplant consolidation in responders; clinical trial preferred.
- 3.Mycosis fungoides / SézaryNCCN category Category 1 (mogamulizumab, brentuximab), NCCN Guidelines: Primary Cutaneous Lymphomas
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.
Asparaginase-based chemotherapy (P-GemOx, SMILE) with involved-site radiotherapy for localised disease; PD-1 inhibitors at relapse.
Pralatrexate, belinostat, romidepsin (ex-US), brentuximab (CD30+), gemcitabine-based regimens, allogeneic HSCT for fit responders.
Generated from this cancer's standard of care, biomarkers and open problems. Take the group that matches where you are. To tick, add your own and print, open the one-page appointment sheet.
Newly diagnosed
- What is my exact diagnosis, stage, and grade, and which tests established them?Everything else follows from an accurate stage and subtype.
- 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?These results decide eligibility for targeted therapy, immunotherapy, and trials.
- Which subtype is my cancer, and does that change the recommended treatment?Recognised subtypes for this cancer include PTCL, not otherwise specified, Nodal TFH lymphoma, ALK-positive ALCL.
- Is germline (inherited) genetic testing recommended for me or my family?Inherited variants can change treatment and matter for relatives.
First line, CD30+ PTCL / ALCL
- For my situation (first line, cd30+ ptcl / alcl), which of the standard options do you recommend and why?Guideline options include: Brentuximab vedotin + CHP ×6 (ECHELON-2, OS benefit) ± consolidative autologous transplant.
- Am I a candidate for Brentuximab vedotin, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
First line, other nodal PTCL
- For my situation (first line, other nodal ptcl), which of the standard options do you recommend and why?Guideline options include: CHOP or CHOEP (≤60 years) ×6, autologous transplant consolidation in responders; clinical trial preferred.
- Am I a candidate for Doxorubicin, Cyclophosphamide, Vincristine, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Relapsed/refractory PTCL
- For my situation (relapsed/refractory ptcl), which of the standard options do you recommend and why?Guideline options include: Pralatrexate, belinostat, romidepsin (ex-US), brentuximab (CD30+), gemcitabine-based regimens, allogeneic HSCT for fit responders.
- Am I a candidate for Pralatrexate, Belinostat, Romidepsin or related drugs, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Mycosis fungoides / Sézary
- For my situation (mycosis fungoides / sézary), which of the standard options do you recommend and 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.
- Am I a candidate for Mogamulizumab, Brentuximab vedotin, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Extranodal NK/T-cell
- For my situation (extranodal nk/t-cell), which of the standard options do you recommend and why?Guideline options include: Asparaginase-based chemotherapy (P-GemOx, SMILE) with involved-site radiotherapy for localised disease; PD-1 inhibitors at relapse.
- Am I a candidate for Pembrolizumab, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Any stage
- Are there clinical trials I could join, for example of Brentuximab vedotin, Azacitidine, Allogeneic stem cell transplantation, Soquelitinib?Trials are how the next standard of care is set; asking early keeps options open.
- Would a second opinion at a high-volume centre change anything, and can you help arrange it?Rare or high-stakes decisions benefit from a centre that treats many similar patients.
- What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?Supportive care improves quality of life and helps patients complete treatment.
- 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?Open problems are where trials and second opinions matter most.
- I read that “Randomised evidence for transplant consolidation is lacking”. How does that affect my plan?Open problems are where trials and second opinions matter most.
Trials open now for this cancer in OnCo, largest phase first. Joining a trial is a decision like any other: ask what the comparison arm is, whether a placebo is used, and what happens if you leave. The cancer page searches ClinicalTrials.gov live for more.
- A Phase 3 Multinational Study of Golidocitinib Versus Investigator's Choice in r/r PTCL (JACKPOT19)Phase 3 · recruiting · NCT07234162A Phase 3, Open-Label, Randomized, Multinational Study to Investigate the Anti-tumor Efficacy of Golidocitinib Versus Investigator's Choice in Adult Patients With Relapsed/Refractory Peripheral T-cell Lymphoma
- A Randomized, Double-blind, Multicenter Phase III Study of XNW5004 Tablets in Patients With Relapsed or Refractory Peripheral T-cell LymphomaPhase 3 · recruiting · NCT06776952A Randomized, Double-blind, Multicenter Phase III Study of XNW5004 Tablets in Patients With Relapsed or Refractory Peripheral T-cell Lymphoma
- Clinical Study of Mitoxantrone Hydrochloride Liposome Injection vs. Chidamide in Patients With Relapsed/Refractory PTCLPhase 3 · active · NCT04668690A Randomized, Open-label, Active Controlled, Multi-center Phase 3 Clinical Study to Compare the Efficacy and Safety of Mitoxantrone Hydrochloride Liposome Injection With Chidamide in Patients With Relapsed/Refractory PTCL.
- 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 LymphomaPhase 3 · recruiting · NCT06561048A Phase 3, Randomized, Open-Label Study to Investigate the Efficacy and Safety of ITK Inhibitor Soquelitinib Versus Physician's Choice Standard of Care Treatment (Selected Single Agent) in Participants With Relapsed/Refractory Peripheral T-cell Lymphoma Not Otherwise Specified, Follicular Helper T-cell Lymphomas, or Systemic Anaplastic Large-cell Lymphoma
- To Evaluate Efficacy of Belinostat or Pralatrexate in Combination Against CHOP Alone in PTCLPhase 3 · recruiting · NCT06072131A Phase 3, Randomized, Open-Label Study Comparing the Efficacy and Safety of the Combination of Beleodaq-CHOP or Folotyn-COP to the CHOP Regimen Alone in Newly Diagnosed Patients With Peripheral T-Cell Lymphoma
Much of what helps in the first weeks is free if you know to ask: testing, helplines, rides and lodging, second opinions, trial travel.
- Peripheral T-cell lymphomas (including cutaneous T-cell lymphoma): the full pagePeripheral 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.
- One-page appointment sheetYour questions, the words you may hear, what to bring, and space for the answers. Print it.
- Treatment sequencingWhich treatment tends to follow which, line by line.
- NavigatorStandard of care for your stage, what you have tried, and trials near you.
- Histologic transformation: When a lung adenocarcinoma escapes targeted therapy by turning into a different cell type, usually small-cell.
- Lugano classification / Ann Arbor staging: The Lugano classification is the lymphoma staging system: stage I to IV by how many lymph node regions and organs are involved, with PET-based response criteria.
Every term links to the glossary.