{"entity":{"id":"lymphoma-ev-randomised-evidence-for-the-t-cell-lymphomas","kind":"idea","name":"Randomised evidence for the T-cell lymphomas, including the ones that are not in Europe or North America","aka":["Randomised trials in peripheral T-cell lymphoma","International trials for NK/T-cell and adult T-cell lymphoma"],"tldr":"Adult T-cell leukaemia has had one randomised trial, in 1998. Most T-cell lymphoma treatment rests on single-arm studies, and the diseases concentrated outside Europe and North America have the least evidence of all.","summary":"The obstacles are structural rather than scientific: no commercial sponsor for diseases concentrated in middle-income countries, regulatory fragmentation across the regions where the diseases occur, and the fact that a trial in a disease with 500 cases a year in any one country can only work internationally. The precedent is the international coordination that made paediatric leukaemia trials possible, which took decades and worked.","asOf":"2026-10-01","links":[],"tags":["lymphoma-evidence"],"related":["lymphoma-roadmap"],"cancers":["peripheral-t-cell-lymphoma","angioimmunoblastic-t-cell-lymphoma","cutaneous-t-cell-lymphoma","non-hodgkin-lymphoma"],"sections":["chemotherapy","immunotherapy"],"technologies":[],"targets":["cd30"],"drugs":["brentuximab-vedotin","asparaginase","cyclophosphamide","doxorubicin","vincristine","prednisone","etoposide"],"companies":[],"institutions":[],"pathways":[],"terms":[],"trials":["jcog9801","smile-enktl","echelon-2","alcanza"],"people":[],"bottlenecks":["b-rare-cancers","b-trial-enrolment","b-global-access","b-regulatory-fragmentation","b-incentive-misalignment"],"keyPapers":["paper-jcog9801-vcap-amp-vecp-adult-t-cell-leukaemia-jco-2007","paper-smile-chemotherapy-nk-t-cell-lymphoma-jco-2011","paper-horwitz-lancet"],"journals":[],"dependsOn":[],"notes":[],"hypothesis":"Internationally coordinated randomised trials in the T-cell lymphomas, run where the diseases are concentrated, would change first-line standards that currently rest on single-arm series.","rationale":"JCOG9801 is the only randomised controlled trial ever run exclusively in adult T-cell leukaemia/lymphoma, with 118 patients, reported in 2007, and its three-year overall survival of 24 per cent in the better arm has not been improved on by a controlled comparison since. SMILE, the regimen that made extranodal NK/T-cell lymphoma treatable, is a 38-patient single-arm phase 2. ECHELON-2 and ALCANZA show that randomised trials in T-cell lymphoma are feasible and change practice when an industry sponsor has a reason to run them. The diseases without a sponsor have no trials.","test":"A standing international platform trial in peripheral T-cell lymphoma with disease-specific strata for adult T-cell leukaemia/lymphoma and extranodal NK/T-cell lymphoma, hosted in Japan, Korea, China, Brazil and the Caribbean as well as in Europe and North America, with a shared control arm and the statistical borrowing that makes rare-disease strata viable.","maturity":"speculative","actor":"policy","cost":"large","horizonYears":10},"route":"/ideas/lymphoma-ev-randomised-evidence-for-the-t-cell-lymphomas/","neighbours":{"roadmap":[{"id":"lymphoma-roadmap","kind":"roadmap","name":"Lymphoma roadmap: from a jaw tumour in Uganda and the first human cancer virus to gene-expression subtypes, PET-adapted chemotherapy, CAR-T cells, bispecific antibodies and the genetics-directed trials now recruiting","route":"/roadmaps/lymphoma-roadmap/"}],"cancer":[{"id":"cutaneous-t-cell-lymphoma","kind":"cancer","name":"Cutaneous T-cell lymphoma (mycosis fungoides and Sezary syndrome)","route":"/cancers/cutaneous-t-cell-lymphoma/"},{"id":"angioimmunoblastic-t-cell-lymphoma","kind":"cancer","name":"Nodal T-follicular helper cell lymphoma, angioimmunoblastic type (angioimmunoblastic T-cell lymphoma)","route":"/cancers/angioimmunoblastic-t-cell-lymphoma/"},{"id":"non-hodgkin-lymphoma","kind":"cancer","name":"Non-Hodgkin lymphoma (all types)","route":"/cancers/non-hodgkin-lymphoma/"},{"id":"peripheral-t-cell-lymphoma","kind":"cancer","name":"Peripheral T-cell lymphomas (including cutaneous T-cell lymphoma)","route":"/cancers/peripheral-t-cell-lymphoma/"}],"section":[{"id":"chemotherapy","kind":"section","name":"Chemotherapy","route":"/fronts/chemotherapy/"},{"id":"immunotherapy","kind":"section","name":"Immunotherapy","route":"/fronts/immunotherapy/"}],"target":[{"id":"cd30","kind":"target","name":"CD30","route":"/targets/cd30/"}],"drug":[{"id":"asparaginase","kind":"drug","name":"Asparaginase (pegaspargase, calaspargase pegol, Erwinia asparaginase)","route":"/drugs/asparaginase/"},{"id":"brentuximab-vedotin","kind":"drug","name":"Brentuximab vedotin","route":"/drugs/brentuximab-vedotin/"},{"id":"cyclophosphamide","kind":"drug","name":"Cyclophosphamide","route":"/drugs/cyclophosphamide/"},{"id":"doxorubicin","kind":"drug","name":"Doxorubicin","route":"/drugs/doxorubicin/"},{"id":"etoposide","kind":"drug","name":"Etoposide","route":"/drugs/etoposide/"},{"id":"prednisone","kind":"drug","name":"Prednisone","route":"/drugs/prednisone/"},{"id":"vincristine","kind":"drug","name":"Vincristine","route":"/drugs/vincristine/"}],"trial":[{"id":"alcanza","kind":"trial","name":"ALCANZA","route":"/trials/alcanza/"},{"id":"echelon-2","kind":"trial","name":"ECHELON-2","route":"/trials/echelon-2/"},{"id":"jcog9801","kind":"trial","name":"JCOG9801","route":"/trials/jcog9801/"},{"id":"smile-enktl","kind":"trial","name":"SMILE","route":"/trials/smile-enktl/"}],"bottleneck":[{"id":"b-incentive-misalignment","kind":"bottleneck","name":"Incentives reward me-too drugs and marginal gains","route":"/bottlenecks/b-incentive-misalignment/"},{"id":"b-global-access","kind":"bottleneck","name":"Most of the world has almost no cancer care","route":"/bottlenecks/b-global-access/"},{"id":"b-rare-cancers","kind":"bottleneck","name":"Rare and paediatric cancers without markets","route":"/bottlenecks/b-rare-cancers/"},{"id":"b-regulatory-fragmentation","kind":"bottleneck","name":"Regulatory divergence between regions","route":"/bottlenecks/b-regulatory-fragmentation/"},{"id":"b-trial-enrolment","kind":"bottleneck","name":"Trials enrol too few, too slowly","route":"/bottlenecks/b-trial-enrolment/"}],"paper":[{"id":"paper-alcanza-brentuximab-vedotin-lancet-2017","kind":"paper","name":"ALCANZA: brentuximab vedotin versus physician's choice in CD30-positive cutaneous T-cell lymphoma","route":"/key-papers/paper-alcanza-brentuximab-vedotin-lancet-2017/"},{"id":"paper-horwitz-lancet","kind":"paper","name":"Brentuximab vedotin with chemotherapy for CD30-positive peripheral T-cell lymphoma (ECHELON-2): a global, double-blind, randomised, phase 3 trial","route":"/key-papers/paper-horwitz-lancet/"},{"id":"paper-smile-chemotherapy-nk-t-cell-lymphoma-jco-2011","kind":"paper","name":"Phase II study of SMILE chemotherapy for newly diagnosed stage IV, relapsed, or refractory extranodal natural killer (NK)/T-cell lymphoma, nasal type: the NK-Cell Tumor Study Group study","route":"/key-papers/paper-smile-chemotherapy-nk-t-cell-lymphoma-jco-2011/"},{"id":"paper-jcog9801-vcap-amp-vecp-adult-t-cell-leukaemia-jco-2007","kind":"paper","name":"VCAP-AMP-VECP compared with biweekly CHOP for adult T-cell leukemia-lymphoma: Japan Clinical Oncology Group Study JCOG9801","route":"/key-papers/paper-jcog9801-vcap-amp-vecp-adult-t-cell-leukaemia-jco-2007/"}]}}