{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T19:42:58Z","timestamp":1790797378447,"version":"4.1.0"},"reference-count":54,"publisher":"MDPI AG","issue":"4","license":[{"start":{"date-parts":[[2015,11,25]],"date-time":"2015-11-25T00:00:00Z","timestamp":1448409600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cancers"],"abstract":"<jats:p>Adult T cell leukemia-lymphoma is a rare disease with a high mortality rate, and is challenging for the clinician. Early allogeneic stem cell transplant can confer durable remission. As novel therapeutic agents become available to treat T cell malignancies, it is increasingly important that medical oncologists, hematologists, and hematopathologists recognize and accurately diagnose adult T cell leukemia-lymphoma. There is no uniform standard of treatment of adult T cell leukemia-lymphoma, and clinical trials remain critical to improving outcomes. Here we present one management approach based on the recent advances in treatment for adult T cell leukemia-lymphoma patients.<\/jats:p>","DOI":"10.3390\/cancers7040893","type":"journal-article","created":{"date-parts":[[2015,11,25]],"date-time":"2015-11-25T10:44:52Z","timestamp":1448448292000},"page":"2318-2329","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":18,"title":["Optimizing Management of Patients with Adult T Cell Leukemia-Lymphoma"],"prefix":"10.3390","volume":"7","author":[{"given":"Jean","family":"Yared","sequence":"first","affiliation":[{"name":"Marlene and Stewart Greenebaum Cancer Center, University of Maryland School of Medicine, 22 S. Greene Street, Baltimore, MD 21204, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amy","family":"Kimball","sequence":"additional","affiliation":[{"name":"Marlene and Stewart Greenebaum Cancer Center, University of Maryland School of Medicine, 22 S. Greene Street, Baltimore, MD 21204, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2015,11,25]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"761","DOI":"10.1172\/JCI115078","article-title":"Molecular biology of the type I human T-cell leukemia virus (HTLV-I) and adult T-cell leukemia","volume":"87","author":"Smith","year":"1991","journal-title":"J. Clin. Investig."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"10638","DOI":"10.1158\/0008-5472.CAN-07-2587","article-title":"Meeting report on the 13th International Conference on Human Retrovirology: Human T-cell leukemia virus research 30 years after adult T-cell leukemia","volume":"67","author":"Matsuoka","year":"2007","journal-title":"Cancer Res."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"250","DOI":"10.1002\/ijc.2910430214","article-title":"Modelling the risk of adult T-cell leukemia\/lymphoma in persons infected with human T-lymphotropic virus type I","volume":"43","author":"Murphy","year":"1989","journal-title":"Int. J. Cancer"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"5473","DOI":"10.1182\/blood-2010-12-327791","article-title":"Clonal evolution of adult T-cell leukemia\/lymphoma takes place in the lymph nodes","volume":"117","author":"Umino","year":"2011","journal-title":"Blood"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"536","DOI":"10.1111\/bjh.12659","article-title":"Differences in incidence and trends of haematological malignancies in Japan and the United States","volume":"164","author":"Chihara","year":"2014","journal-title":"Br. J. Haematol."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"4795","DOI":"10.1182\/blood-2012-07-440073","article-title":"Classification of non-Hodgkin lymphoma in Central and South America: A review of 1028 cases","volume":"120","author":"Laurini","year":"2012","journal-title":"Blood"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"381","DOI":"10.1089\/aid.1993.9.381","article-title":"An HTLV-I vaccine: Why, how, for whom?","volume":"9","author":"Bomford","year":"1993","journal-title":"AIDS Res. Hum. Retrovir."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"1685","DOI":"10.1182\/blood.V124.21.1685.1685","article-title":"Analysis of Large Cohort Shows That Caribbean Adult T Cell Leukemia\/Lymphoma Is a Chemotherapy Refractory Disease with Very Poor Prognosis That Behaves Distinctly from Japanese Subtypes","volume":"124","author":"Zell","year":"2014","journal-title":"Blood"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"4500","DOI":"10.1182\/blood-2005-09-3801","article-title":"Identification of subtype-specific genomic alterations in aggressive adult T-cell leukemia\/lymphoma","volume":"107","author":"Oshiro","year":"2006","journal-title":"Blood"},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"375","DOI":"10.1046\/j.1365-2141.2001.02737.x","article-title":"A new G-CSF-supported combination chemotherapy, LSG15, for adult T-cell leukaemia-lymphoma: Japan Clinical Oncology Group Study 9303","volume":"113","author":"Yamada","year":"2001","journal-title":"Br. J. Haematol."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"4177","DOI":"10.1200\/JCO.2010.28.0669","article-title":"Meta-analysis on the use of zidovudine and interferon-alfa in adult T-cell leukemia\/lymphoma showing improved survival in the leukemic subtypes","volume":"28","author":"Bazarbachi","year":"2010","journal-title":"J. Clin. Oncol."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1046\/j.1440-1827.2002.01308.x","article-title":"The World Health Organization classification of malignant lymphoma: Incidence and clinical prognosis in HTLV-1-endemic area of Fukuoka","volume":"52","author":"Ohshima","year":"2002","journal-title":"Pathol. Int."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"428","DOI":"10.1111\/j.1365-2141.1991.tb08051.x","article-title":"Diagnostic criteria and classification of clinical subtypes of adult T-cell leukaemia-lymphoma. A report from the Lymphoma Study Group (1984\u201387)","volume":"79","author":"Shimoyama","year":"1991","journal-title":"Br. J. Haematol."},{"key":"ref_14","unstructured":"Swerdlow, S.H. (2008). WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues, International Agency for Research on Cancer, World Health Organization."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"e517","DOI":"10.1016\/S1470-2045(14)70202-5","article-title":"Human T-cell leukaemia virus type I and adult T-cell leukaemia-lymphoma","volume":"15","author":"Ishitsuka","year":"2014","journal-title":"Lancet Oncol."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"3048","DOI":"10.1200\/JCO.2013.53.5229","article-title":"Role of imaging in the staging and response assessment of lymphoma: Consensus of the International Conference on Malignant Lymphomas Imaging Working Group","volume":"32","author":"Barrington","year":"2014","journal-title":"J. Clin. Oncol."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"4337","DOI":"10.1182\/blood-2009-09-242347","article-title":"Long-term study of indolent adult T-cell leukemia-lymphoma","volume":"115","author":"Takasaki","year":"2010","journal-title":"Blood"},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"52","DOI":"10.1186\/1742-4690-10-52","article-title":"Interferon-alpha (IFN-alpha) suppresses HTLV-1 gene expression and cell cycling, while IFN-alpha combined with zidovudine induces p53 signaling and apoptosis in HTLV-1-infected cells","volume":"10","author":"Kinpara","year":"2013","journal-title":"Retrovirology"},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"5458","DOI":"10.1200\/JCO.2007.11.9958","article-title":"VCAP-AMP-VECP compared with biweekly CHOP for adult T-cell leukemia-lymphoma: Japan Clinical Oncology Group Study JCOG9801","volume":"25","author":"Tsukasaki","year":"2007","journal-title":"J. Clin. Oncol."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"779","DOI":"10.1046\/j.1365-2141.2001.02794.x","article-title":"Interferon alpha and zidovudine therapy in adult T-cell leukaemia lymphoma: Response and outcome in 15 patients","volume":"113","author":"Matutes","year":"2001","journal-title":"Br. J. Haematol."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"6528","DOI":"10.1182\/blood-2009-03-211821","article-title":"Phase 2 study of the efficacy and safety of the combination of arsenic trioxide, interferon alpha, and zidovudine in newly diagnosed chronic adult T-cell leukemia\/lymphoma (ATL)","volume":"113","author":"Kchour","year":"2009","journal-title":"Blood"},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"1744","DOI":"10.1056\/NEJM199506293322603","article-title":"Treatment of adult T-cell leukemia-lymphoma with a combination of interferon alfa and zidovudine","volume":"332","author":"Gill","year":"1995","journal-title":"N. Engl. J. Med."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"276","DOI":"10.1038\/sj.thj.6200195","article-title":"A prospective phase II clinical trial with the use of zidovudine and interferon-alpha in the acute and lymphoma forms of adult T-cell leukemia\/lymphoma","volume":"3","author":"Hermine","year":"2002","journal-title":"Hematol. J."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"85","DOI":"10.1093\/jjco\/hyr168","article-title":"Lymphoma study group of JCOG","volume":"42","author":"Tsukasaki","year":"2012","journal-title":"Jpn. J. Clin. Oncol."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"4696","DOI":"10.1200\/JCO.2011.35.5578","article-title":"Use of zidovudine and interferon alfa with chemotherapy improves survival in both acute and lymphoma subtypes of adult T-cell leukemia\/lymphoma","volume":"29","author":"Hodson","year":"2011","journal-title":"J. Clin. Oncol."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"e4420","DOI":"10.1371\/journal.pone.0004420","article-title":"Human T cell leukemia virus reactivation with progression of adult T-cell leukemia-lymphoma","volume":"4","author":"Ratner","year":"2009","journal-title":"PLoS ONE"},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"1816","DOI":"10.1182\/blood-2012-01-401349","article-title":"Radiation therapy for the management of patients with HTLV-1-associated adult T-cell leukemia\/lymphoma","volume":"120","author":"Simone","year":"2012","journal-title":"Blood"},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"453","DOI":"10.1200\/JCO.2008.18.2428","article-title":"Definition, prognostic factors, treatment, and response criteria of adult T-cell leukemia-lymphoma: A proposal from an international consensus meeting","volume":"27","author":"Tsukasaki","year":"2009","journal-title":"J. Clin. Oncol."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"e159","DOI":"10.1038\/bcj.2013.57","article-title":"Association between decreasing trend in the mortality of adult T-cell leukemia\/lymphoma and allogeneic hematopoietic stem cell transplants in Japan: Analysis of Japanese vital statistics and Japan Society for Hematopoietic Cell Transplantation (JSHCT)","volume":"3","author":"Chihara","year":"2013","journal-title":"Blood Cancer J."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"1039","DOI":"10.1080\/10428190902887571","article-title":"A multi-institutional experience of autologous stem cell transplantation in North American patients with human T-cell lymphotropic virus type-1 adult T-cell leukemia\/lymphoma suggests ineffective salvage of relapsed patients","volume":"50","author":"Phillips","year":"2009","journal-title":"Leuk. Lymphoma"},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"1029","DOI":"10.1038\/bmt.2008.39","article-title":"Graft-versus-adult T-cell leukemia\/lymphoma effect following allogeneic hematopoietic stem cell transplantation","volume":"41","author":"Yonekura","year":"2008","journal-title":"Bone Marrow Transplant."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"4143","DOI":"10.1182\/blood-2004-11-4193","article-title":"Allogeneic stem-cell transplantation with reduced conditioning intensity as a novel immunotherapy and antiviral therapy for adult T-cell leukemia\/lymphoma","volume":"105","author":"Okamura","year":"2005","journal-title":"Blood"},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"391","DOI":"10.1158\/0008-5472.CAN-03-1452","article-title":"Graft-versus-Tax response in adult T-cell leukemia patients after hematopoietic stem cell transplantation","volume":"64","author":"Harashima","year":"2004","journal-title":"Cancer Res."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"817","DOI":"10.1016\/j.bbmt.2008.04.014","article-title":"A retrospective analysis of allogeneic hematopoietic stem cell transplantation for adult T cell leukemia\/lymphoma (ATL): Clinical impact of graft-versus-leukemia\/lymphoma effect","volume":"14","author":"Shiratori","year":"2008","journal-title":"Biol. Blood Marrow Transplant."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"1731","DOI":"10.1016\/j.bbmt.2013.09.014","article-title":"Impact of graft-versus-host disease on allogeneic hematopoietic cell transplantation for adult T cell leukemia-lymphoma focusing on preconditioning regimens: Nationwide retrospective study","volume":"19","author":"Ishida","year":"2013","journal-title":"Biol. Blood Marrow Transplant."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"116","DOI":"10.1038\/bmt.2010.92","article-title":"Long-term outcomes after hematopoietic SCT for adult T-cell leukemia\/lymphoma: Results of prospective trials","volume":"46","author":"Choi","year":"2011","journal-title":"Bone Marrow Transplant."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"1340","DOI":"10.1007\/s10875-012-9729-5","article-title":"Long-term persistence of limited HTLV-I Tax-specific cytotoxic T cell clones in a patient with adult T cell leukemia\/lymphoma after allogeneic stem cell transplantation","volume":"32","author":"Tanaka","year":"2012","journal-title":"J. Clin. Immunol."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"1369","DOI":"10.1182\/blood-2009-10-247510","article-title":"Transplantation of allogeneic hematopoietic stem cells for adult T-cell leukemia: A nationwide retrospective study","volume":"116","author":"Hishizawa","year":"2010","journal-title":"Blood"},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"696","DOI":"10.1016\/j.bbmt.2014.12.020","article-title":"A Retrospective Analysis of Treatment Outcomes in Adult T Cell Leukemia\/Lymphoma Patients with Aggressive Disease Treated with or without Allogeneic Stem Cell Transplantation: A Single-Center Experience","volume":"21","author":"Kawada","year":"2015","journal-title":"Biol. Blood Marrow Transplant."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"2141","DOI":"10.1182\/blood-2011-07-368233","article-title":"Impact of graft-versus-host disease on outcomes after allogeneic hematopoietic cell transplantation for adult T-cell leukemia: A retrospective cohort study","volume":"119","author":"Kanda","year":"2012","journal-title":"Blood"},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"801","DOI":"10.1016\/j.bbmt.2014.02.014","article-title":"High level of serum soluble interleukin-2 receptor at transplantation predicts poor outcome of allogeneic stem cell transplantation for adult T cell leukemia","volume":"20","author":"Shigematsu","year":"2014","journal-title":"Biol. Blood Marrow Transplant."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"1734","DOI":"10.1182\/blood-2012-03-414490","article-title":"Allogeneic hematopoietic stem cell transplantation for adult T-cell leukemia-lymphoma with special emphasis on preconditioning regimen: A nationwide retrospective study","volume":"120","author":"Ishida","year":"2012","journal-title":"Blood"},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"1124","DOI":"10.1016\/S0140-6736(11)60315-2","article-title":"Donor-derived adult T-cell leukaemia","volume":"377","author":"Nakamizo","year":"2011","journal-title":"Lancet"},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"1758","DOI":"10.1056\/NEJMc053295","article-title":"Donor-derived T-cell leukemia after bone marrow transplantation","volume":"354","author":"Tamaki","year":"2006","journal-title":"N. Engl. J. Med."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"1968","DOI":"10.1016\/j.bbmt.2014.08.012","article-title":"Treatment of patients with adult T cell leukemia\/lymphoma with cord blood transplantation: A Japanese nationwide retrospective survey","volume":"20","author":"Kato","year":"2014","journal-title":"Biol. Blood Marrow Transplant."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"3108","DOI":"10.1182\/blood-2006-09-046219","article-title":"Extended follow-up of methotrexate-free immunosuppression using sirolimus and tacrolimus in related and unrelated donor peripheral blood stem cell transplantation","volume":"109","author":"Cutler","year":"2007","journal-title":"Blood"},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"993","DOI":"10.1002\/ijc.25742","article-title":"Inhibition of mammalian target of rapamycin signaling by everolimus induces senescence in adult T-cell leukemia\/lymphoma and apoptosis in peripheral T-cell lymphomas","volume":"129","author":"Darwiche","year":"2011","journal-title":"Int. J. Cancer"},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"219","DOI":"10.1182\/blood-2012-07-444372","article-title":"Treatment of relapsed adult T-cell leukemia\/lymphoma after allogeneic hematopoietic stem cell transplantation: The Nagasaki Transplant Group experience","volume":"121","author":"Itonaga","year":"2013","journal-title":"Blood"},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"725","DOI":"10.1007\/s12185-012-1056-3","article-title":"Successful treatment by donor lymphocyte infusion of adult T-cell leukemia\/lymphoma relapse following allogeneic hematopoietic stem cell transplantation","volume":"95","author":"Kamimura","year":"2012","journal-title":"Int. J. Hematol."},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"1293","DOI":"10.2165\/11631090-000000000-00000","article-title":"Mogamulizumab: First global approval","volume":"72","author":"Subramaniam","year":"2012","journal-title":"Drugs"},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"837","DOI":"10.1200\/JCO.2011.37.3472","article-title":"Defucosylated anti-CCR4 monoclonal antibody (KW-0761) for relapsed adult T-cell leukemia-lymphoma: A multicenter phase II study","volume":"30","author":"Ishida","year":"2012","journal-title":"J. Clin. Oncol."},{"key":"ref_52","first-page":"60","article-title":"Brentuximab vedotin administered concurrently with multi-agent chemotherapy as frontline treatment of ALCL and other CD30-positive mature T-cell and NK-cell lymphomas","volume":"120","author":"Fanale","year":"2012","journal-title":"ASH Annu. Meet. Abstr."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"1357","DOI":"10.1038\/sj.leu.2403400","article-title":"Proteasome inhibitor, bortezomib, potently inhibits the growth of adult T-cell leukemia cells both in vivo and in vitro","volume":"18","author":"Satou","year":"2004","journal-title":"Leukemia"},{"key":"ref_54","first-page":"2737","article-title":"Multicenter Phase I Dose-Escalation Study of Lenalidomide in Patients with Relapsed Adult T-Cell Leukemia-Lymphoma (ATL) or Peripheral T-Cell Lymphoma (PTCL)","volume":"120","author":"Uike","year":"2012","journal-title":"ASH Annu. Meet. Abstr."}],"container-title":["Cancers"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2072-6694\/7\/4\/893\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T20:52:42Z","timestamp":1760215962000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2072-6694\/7\/4\/893"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2015,11,25]]},"references-count":54,"journal-issue":{"issue":"4","published-online":{"date-parts":[[2015,12]]}},"alternative-id":["cancers7040893"],"URL":"https:\/\/doi.org\/10.3390\/cancers7040893","relation":{},"ISSN":["2072-6694"],"issn-type":[{"value":"2072-6694","type":"electronic"}],"subject":[],"published":{"date-parts":[[2015,11,25]]}}}