• HTLV-1, Tax and HBZ in adult T-cell leukaemia/lymphoma: A virus passed mostly from mother to child in breast milk, common in parts of Japan, the Caribbean, west Africa and South America.
• HTLV-1 (human T-lymphotropic virus type 1): A virus that infects T cells, is passed mainly through breastfeeding and sexual contact, and causes a lymphoma decades later in a small minority of the people it infects.
• Lymphoma treatments that did not work: the negative trials worth knowing: A list of things that were expected to improve lymphoma treatment and did not.
• Epstein-Barr virus latency programmes, and why they decide which lymphoma: Almost everyone carries Epstein-Barr virus for life without harm.
• What it costs to aim at a lineage antigen: Almost every lymphoma drug that finds the cancer by a surface marker finds healthy cells carrying the same marker.
• Lymphoma (tissue type): Cancer of lymphocytes, the white blood cells of the immune system, usually growing as masses in lymph nodes, spleen or other organs.
• Plasma EBV DNA: Fragments of Epstein-Barr virus DNA in the blood that measure nasopharyngeal carcinoma: used to screen healthy people in endemic regions, to stage, to decide who needs extra treatment after radiotherapy, and to detect relapse.
• Vaccinations around lymphoma treatment: the ones to have first, and the ones not to have at all: Inactivated vaccines work best when they are given at least two weeks before immunosuppressive treatment begins, and live vaccines are generally not given to someone whose immune system is suppressed.
• The surveillance schedule, and the evidence that routine scans do not find relapse first: Most people expect regular scans after lymphoma treatment and are unsettled when they are not offered.
• Fertility preservation before lymphoma treatment: a decision with a deadline in days: Most of the ways of preserving fertility have to happen before the first dose of chemotherapy, and two of them take about a fortnight.

Biomarker results to ask for: 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.

Scans and tests linked to this cancer: Comprehensive genomic profiling, FDG PET, Histopathology & immunohistochemistry, Multidisciplinary tumour boards, Multiparameter flow cytometry MRD.

Bring copies of scan reports, pathology and blood results, and a list of every medicine and supplement.

• First line, CD30+ PTCL / ALCL: Brentuximab vedotin + CHP ×6 (ECHELON-2, OS benefit) ± consolidative autologous transplant. (Brentuximab vedotin, Autologous 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. (Doxorubicin, Cyclophosphamide, Vincristine, Autologous stem cell transplant (high-dose therapy))
• 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, Brentuximab vedotin, Allogeneic 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. (Pembrolizumab, IMRT / IGRT (modern external beam))
• Peripheral T-cell lymphoma: the questions that decide the regimen: Four things to establish before treatment. Which entity: ALK-positive anaplastic large cell lymphoma does much better than every other nodal T-cell lymphoma and is curable with chemotherapy alone in most patients; ALK-negative anaplastic large cell lymphoma, angioimmunoblastic T-cell lymphoma, peripheral T-cell lymphoma not otherwise specified and the enteropathy-associated forms do considerably worse. CD30 expression by immunohistochemistry, because it decides whether brentuximab vedotin is added to first-line chemotherapy. Whether the disease is nodal, extranodal (nasal NK/T-cell), leukaemic (adult T-cell leukaemia/lymphoma, T-cell prolymphocytic leukaemia) or cutaneous, because those four groups have entirely different treatments. HTLV-1 serology where the patient comes from or has family from Japan, the Caribbean, west Africa, Iran or parts of South America, because adult T-cell leukaemia/lymphoma is treated differently from everything else. A breast implant-associated anaplastic large cell lymphoma presenting as a late seroma around an implant is treated primarily by complete surgical removal of the implant and capsule, and most patients need nothing else. That is a different disease from systemic anaplastic large cell lymphoma despite the shared name. (Lymphoma (tissue type), The lymphoma regimen alphabet: R-CHOP, pola-R-CHP, DA-EPOCH-R, ABVD, BEACOPP and the rest, Brentuximab vedotin, FDG PET, Lugano classification / Ann Arbor staging)
• First-line nodal peripheral T-cell lymphoma, CD30-positive: brentuximab vedotin with CHP: ECHELON-2 randomised 452 patients with untreated CD30-positive peripheral T-cell lymphoma to brentuximab vedotin with cyclophosphamide, doxorubicin and prednisone (A+CHP) or to CHOP. Median progression-free survival by blinded independent review was 48.2 against 20.8 months (hazard ratio 0.71), with improved overall survival, and toxicity was comparable: febrile neutropenia 18 against 15 per cent and peripheral neuropathy 52 against 55 per cent. It is the only positive randomised first-line trial in this family in twenty years and it changed the standard. Three-quarters of the trial population had systemic anaplastic large cell lymphoma, so the benefit is best established there and is extrapolated to other CD30-positive entities. Six cycles are given, with G-CSF support. Vincristine is omitted because brentuximab vedotin is itself a tubulin-directed agent and giving both causes unacceptable neuropathy. (ECHELON-2, Brentuximab vedotin, Cyclophosphamide, Doxorubicin, Prednisone, Growth factors: G-CSF and febrile neutropenia prevention, The lymphoma regimen alphabet: R-CHOP, pola-R-CHP, DA-EPOCH-R, ABVD, BEACOPP and the rest, Brentuximab vedotin with chemotherapy for CD30-positive peripheral T-cell lymphoma (ECHELON-2): a global, double-blind, randomised, phase 3 trial)
• First-line nodal peripheral T-cell lymphoma, CD30-negative: CHOP, CHOEP and an honest account of the evidence: CHOP for six cycles is the default, and it is a default rather than a demonstrated best. No randomised trial has shown any regimen superior to CHOP in CD30-negative nodal peripheral T-cell lymphoma, and roughly half of patients are not cured by it. Adding etoposide (CHOEP) is standard in much of Europe for patients under 60 with a normal LDH, on the basis of a retrospective analysis of the German High-Grade Non-Hodgkin Lymphoma Study Group trials in which that subgroup had better event-free survival; the analysis was not randomised and not confined to T-cell lymphoma, so the practice rests on an observation rather than a trial. Adding romidepsin to CHOP was tested properly in the Ro-CHOP trial and failed, which is why the United States withdrew romidepsin's peripheral T-cell lymphoma indication. Angioimmunoblastic T-cell lymphoma is the entity in which the epigenetic drugs look most promising, because it is driven by mutations in TET2, DNMT3A, IDH2 and RHOA; azacitidine-containing combinations and histone deacetylase inhibitors are being tested and are not yet standard. Enteropathy-associated T-cell lymphoma is treated with intensive regimens and early nutritional support, because it presents with perforation or obstruction in a malnourished patient with coeliac disease. Entering a trial is a reasonable first choice rather than a last resort in this group. (R-CHOP (lymphoma chemoimmunotherapy), Cyclophosphamide, Doxorubicin, Vincristine, Prednisone, Etoposide, Romidepsin, The lymphoma regimen alphabet: R-CHOP, pola-R-CHP, DA-EPOCH-R, ABVD, BEACOPP and the rest, Lymphoma treatments that did not work: the negative trials worth knowing)
• Autologous transplant consolidation in first remission: Consolidating a first complete or partial remission with high-dose therapy and an autologous stem cell transplant is standard practice in Europe and common in the United States for nodal peripheral T-cell lymphomas other than ALK-positive anaplastic large cell lymphoma, which does well enough without it. The evidence is a prospective single-arm Nordic study and a series of registry comparisons; there has never been a randomised trial, and the registry comparisons are subject to the obvious bias that only patients who respond well enough and stay fit enough reach transplant. So the row is written as it is: this is the convention, it is reasonable, and a patient is entitled to be told that its benefit has not been demonstrated against continuing observation. Stem cells are collected after two to four cycles, before the marrow is exhausted. Allogeneic transplant is reserved for relapsed disease and for hepatosplenic T-cell lymphoma, where it is the only treatment that produces long remissions. (Autologous stem cell transplant (high-dose therapy), Allogeneic stem cell transplantation, Stem cell transplant in lymphoma: what it is still for, Carmustine, Etoposide, Cytarabine, Melphalan (including hepatic delivery system))
• Relapsed/refractory PTCL: Pralatrexate, belinostat, romidepsin (ex-US), brentuximab (CD30+), gemcitabine-based regimens, allogeneic HSCT for fit responders. (Pralatrexate, Belinostat, Romidepsin, Brentuximab vedotin, Allogeneic stem cell transplantation)
• Relapsed and refractory peripheral T-cell lymphoma: Response rates to every available single agent sit between about 25 and 35 per cent, and remissions are usually short, so the question at relapse is whether the patient can be taken to an allogeneic transplant or a trial. Approved single agents in the United States: pralatrexate, an antifolate, approved in 2009 on a response rate of about 29 per cent; belinostat, a histone deacetylase inhibitor, approved in 2014 on about 26 per cent, usable when platelets are low; romidepsin, approved in 2011 and withdrawn from this indication after the confirmatory Ro-CHOP trial failed. Brentuximab vedotin is highly active in relapsed systemic anaplastic large cell lymphoma, where response rates are far higher than in other entities; its first-line United States approval with cyclophosphamide, doxorubicin and prednisone for untreated systemic anaplastic large cell lymphoma and other CD30-expressing peripheral T-cell lymphomas came on 16 November 2018. Availability differs sharply between countries: pralatrexate and belinostat are not routinely commissioned in England. Other options are gemcitabine-based chemotherapy (GemOx, GDP), which is widely used in the United Kingdom and has no randomised support, bendamustine, alemtuzumab in selected cases, and a clinical trial. Allogeneic transplant is the only treatment with curative potential at this point and is offered to fit patients who achieve a response. Valemetostat, an EZH1/EZH2 inhibitor, is approved in Japan for relapsed adult T-cell leukaemia/lymphoma and peripheral T-cell lymphoma and is in trials elsewhere; the corpus does not yet hold a record for it and this page does not quote a figure for it. (Pralatrexate, Belinostat, Romidepsin, Brentuximab vedotin, Gemcitabine, Oxaliplatin, Bendamustine, Alemtuzumab, Allogeneic stem cell transplantation, Stem cell transplant in lymphoma: what it is still for)

From the standard of care recorded for this cancer; which apply depends on your stage and biomarkers. Ask which the team recommends and why.