{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T23:24:04Z","timestamp":1791329044636,"version":"4.3.1"},"reference-count":42,"publisher":"BMJ","issue":"12","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J Clin Pathol"],"published-print":{"date-parts":[[2007,12]]},"abstract":"<jats:p>Adult T-cell leukaemia\/lymphoma (ATLL) is a mature T-cell neoplasm of post-thymic lymphocytes aetiologically linked to the human T-cell lymphotropic virus, HTLV-I, and with a distinct geographical distribution. The disease manifests with leukaemia in greater than two thirds of patients, while the remaining patients have a lymphomatous form. According to the disease manifestations, various forms which differ in clinical course and prognosis have been recognised: acute, chronic, smouldering and lymphoma. Organomegaly, skin involvement, circulating atypical lymphocytes (\u201cflower\u201d cells) with a CD4+ CD25+ phenotype and hypercalcaemia are the most common disease features. The diagnosis should be based on a constellation of clinical features and laboratory investigations. The latter comprise: lymphocyte morphology, immunophenotype, histology of the tissues affected in the pure lymphoma forms and serology or DNA analysis for HTLV-I. The differential diagnosis of ATLL includes other mature T-cell neoplasms such as T-cell prolymphocytic leukaemia (T-PLL), S\u00e9zary syndrome (SS), peripheral T-cell lymphomas and occasionally healthy carriers of the virus or Hodgkin disease. The clinical course is aggressive with a median survival of less than 12 months in the acute and lymphoma forms. Despite major advances in understanding the pathogenesis of the disease, management of these patients remains a challenge for clinicians as they do not respond or achieve only transient responses to therapies used in high-grade lymphomas. The use of antiretroviral agents such as zidovudine in combination with interferon-alpha, with or without concomitant chemotherapy, has shown activity in this disease with improvement in survival and response rate. Consolidation with high dose therapy and autologous or allogeneic stem-cell transplantation should be considered in young patients.<\/jats:p>","DOI":"10.1136\/jcp.2007.052456","type":"journal-article","created":{"date-parts":[[2007,11,27]],"date-time":"2007-11-27T13:23:11Z","timestamp":1196169791000},"page":"1373-1377","source":"Crossref","is-referenced-by-count":154,"title":["Adult T-cell leukaemia\/lymphoma"],"prefix":"10.1136","volume":"60","author":[{"given":"E","family":"Matutes","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2007,11,27]]},"reference":[{"key":"2025112110140383000_60.12.1373.1","unstructured":"Kikuchi M Jaffe ES Ralfkiaer E . Adult T-cell leukaemia\/lymphoma. In: Jaffe ES Harris NL Stein H Vardiman JW , eds. World Health Organization classification of tumours. Tumours of haematopoietic and lymphoid tissues. Lyon: IARC Press, 2001:200\u00e2\u0080\u00933."},{"key":"2025112110140383000_60.12.1373.2","unstructured":"Matutes E Catovsky D . Adult T cell leukaemia lymphoma. In: Whittaker JA , eds. Leukaemia, 3rd edn. Oxford: Blackwell Scientific Publications, 1998:416\u00e2\u0080\u009333."},{"key":"2025112110140383000_60.12.1373.3","doi-asserted-by":"crossref","first-page":"428","DOI":"10.1111\/j.1365-2141.1991.tb08051.x","article-title":"Diagnostic criteria and clinical subtypes of ATLL. A report from the Lymphoma Study Group (1984\u00e2\u0080\u009387).","volume":"79","author":"Shimomaya","year":"1991","journal-title":"Br J Haematol"},{"key":"2025112110140383000_60.12.1373.4","doi-asserted-by":"publisher","DOI":"10.1016\/0140-6736(91)90737-A"},{"key":"2025112110140383000_60.12.1373.5","doi-asserted-by":"crossref","first-page":"637","DOI":"10.1080\/10428199909169632","article-title":"HTLV-I induced intestinal lymphoma.","volume":"35","author":"Cabrera","year":"1999","journal-title":"Leuk Lymphoma"},{"key":"2025112110140383000_60.12.1373.6","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.2005.05629.x"},{"key":"2025112110140383000_60.12.1373.7","doi-asserted-by":"publisher","DOI":"10.1136\/jcp.2007.052662"},{"key":"2025112110140383000_60.12.1373.8","doi-asserted-by":"publisher","DOI":"10.1016\/0140-6736(90)92432-H"},{"key":"2025112110140383000_60.12.1373.9","doi-asserted-by":"publisher","DOI":"10.1002\/1097-0142(19851001)56:7<1688::AID-CNCR2820560736>3.0.CO;2-O"},{"key":"2025112110140383000_60.12.1373.10","doi-asserted-by":"crossref","first-page":"615","DOI":"10.1111\/j.1365-2141.1995.tb08371.x","article-title":"The first report of familial adult T-cell leukaemia lymphoma in the United Kingdom.","volume":"89","author":"Matutes","year":"1995","journal-title":"Br J Haematol"},{"key":"2025112110140383000_60.12.1373.11","doi-asserted-by":"crossref","first-page":"459","DOI":"10.3109\/10428199509056858","article-title":"HTLV-I positive spastic paraparesis (TSP) associated with a lymphoid disorder in three Chilean patients.","volume":"17","author":"Cartier","year":"1995","journal-title":"Leuk Lymphoma"},{"key":"2025112110140383000_60.12.1373.12","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.20065"},{"key":"2025112110140383000_60.12.1373.13","doi-asserted-by":"crossref","first-page":"94","DOI":"10.1016\/S0140-6736(87)92752-8","article-title":"Strongyloides stercoralis as candidate cofactor for HTLV-I induced malignancies.","volume":"ii","author":"Yamaguchi","year":"1987","journal-title":"Lancet"},{"key":"2025112110140383000_60.12.1373.14","doi-asserted-by":"crossref","first-page":"1027","DOI":"10.1182\/blood.V71.4.1027.1027","article-title":"HTLV-I induced lymphoma mimicking Hodgkin\u00e2\u0080\u0099s disease. Diagnosis by polymerase chain reaction amplification of specific HTLV-I sequences in tumor DNA.","volume":"71","author":"Duggan","year":"1988","journal-title":"Blood"},{"key":"2025112110140383000_60.12.1373.15","doi-asserted-by":"publisher","DOI":"10.1002\/1097-0142(19910315)67:6<1622::AID-CNCR2820670625>3.0.CO;2-1"},{"key":"2025112110140383000_60.12.1373.16","doi-asserted-by":"publisher","DOI":"10.1002\/ijc.2910350111"},{"key":"2025112110140383000_60.12.1373.17","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.1998.00761.x"},{"key":"2025112110140383000_60.12.1373.18","doi-asserted-by":"publisher","DOI":"10.1016\/0145-2126(85)90122-5"},{"key":"2025112110140383000_60.12.1373.19","first-page":"1554","article-title":"Correlation of chromosome abnormalities with histologic and immunologic characteristics in non-Hodgkin\u00e2\u0080\u0099s lymphoma and adult T-cell leukemia lymphoma.","volume":"70","year":"1988","journal-title":"Blood"},{"key":"2025112110140383000_60.12.1373.20","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(94)90994-6"},{"key":"2025112110140383000_60.12.1373.21","first-page":"43","article-title":"Treatment of patients with adult T-cell leukemia-lymphoma: an overview.","volume-title":"Advances in adult T-cell leukemia and HTLV-I research","volume":"39","author":"Shimoyama","year":"1992"},{"key":"2025112110140383000_60.12.1373.22","first-page":"594","article-title":"Clinical significance of B2-microglobulin in serum of adult T cell leukemia.","volume":"9","author":"Sadamori","year":"1995","journal-title":"Leukemia"},{"key":"2025112110140383000_60.12.1373.23","first-page":"598","article-title":"P53 gene mutation and loss of heterozygosity are associated with increased risk of disease progression in adult T cell leukemia.","volume":"9","author":"Nishimura","year":"1995","journal-title":"Leukemia"},{"key":"2025112110140383000_60.12.1373.24","first-page":"1834","article-title":"A new classification of clinical stages of adult T-cell leukemia based on prognosis of the disease.","volume":"8","author":"Shirono","year":"1994","journal-title":"Leukemia"},{"key":"2025112110140383000_60.12.1373.25","doi-asserted-by":"publisher","DOI":"10.1016\/S1470-2045(04)01608-0"},{"key":"2025112110140383000_60.12.1373.26","doi-asserted-by":"publisher","DOI":"10.1038\/sj.onc.1208979"},{"key":"2025112110140383000_60.12.1373.27","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.2001.02737.x"},{"key":"2025112110140383000_60.12.1373.28","doi-asserted-by":"crossref","first-page":"2588","DOI":"10.1200\/JCO.1994.12.12.2588","article-title":"The role of pentostatin in the treatment of T-cell malignancies: analysis of response rate in 145 patients according to disease subtype.","volume":"12","author":"Mercieca","year":"1994","journal-title":"J Clin Oncol"},{"key":"2025112110140383000_60.12.1373.29","doi-asserted-by":"crossref","first-page":"1701","DOI":"10.1182\/blood.V82.6.1701.1701","article-title":"The interleukin-2 receptor: a target for monoclonal antibody treatment of human T-cell lymphotropic virus-I induced adult T-cell leukemia.","volume":"82","author":"Waldmann","year":"1993","journal-title":"Blood"},{"key":"2025112110140383000_60.12.1373.30","doi-asserted-by":"crossref","first-page":"4063","DOI":"10.1182\/blood.V86.11.4063.bloodjournal86114063","article-title":"Radio-immunotherapy of interleukin-2R alpha expressing adult T-cell leukemia with yttrium-90-labeled anti-Tac.","volume":"86","author":"Waldmann","year":"1995","journal-title":"Blood"},{"key":"2025112110140383000_60.12.1373.31","doi-asserted-by":"publisher","DOI":"10.1016\/j.leukres.2005.05.008"},{"key":"2025112110140383000_60.12.1373.32","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2005-01-0335"},{"key":"2025112110140383000_60.12.1373.33","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199506293322603"},{"key":"2025112110140383000_60.12.1373.34","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199506293322604"},{"key":"2025112110140383000_60.12.1373.35","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.2001.02794.x"},{"key":"2025112110140383000_60.12.1373.36","doi-asserted-by":"publisher","DOI":"10.1038\/sj.thj.6200195"},{"key":"2025112110140383000_60.12.1373.37","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2006-05-024604"},{"key":"2025112110140383000_60.12.1373.38","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2006-01-0067"},{"key":"2025112110140383000_60.12.1373.39","doi-asserted-by":"publisher","DOI":"10.1038\/sj.bmt.1702731"},{"key":"2025112110140383000_60.12.1373.40","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.2003.04054.x"},{"key":"2025112110140383000_60.12.1373.41","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1038\/sj.bmt.1705197","article-title":"Bone marrow transplantation from unrelated donors for patients with adult T-cell leukaemia\/lymphoma.","volume":"37","author":"Nakase","year":"2006","journal-title":"Bone Marrow Transplant"},{"key":"2025112110140383000_60.12.1373.42","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2004-11-4193"}],"container-title":["Journal of Clinical Pathology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/jcp.2007.052456","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,11,21]],"date-time":"2025-11-21T18:14:17Z","timestamp":1763748857000},"score":1,"resource":{"primary":{"URL":"https:\/\/jcp.bmj.com\/lookup\/doi\/10.1136\/jcp.2007.052456"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2007,11,27]]},"references-count":42,"journal-issue":{"issue":"12","published-online":{"date-parts":[[2007,11,27]]},"published-print":{"date-parts":[[2007,12]]}},"alternative-id":["10.1136\/jcp.2007.052456"],"URL":"https:\/\/doi.org\/10.1136\/jcp.2007.052456","relation":{},"ISSN":["0021-9746","1472-4146"],"issn-type":[{"value":"0021-9746","type":"print"},{"value":"1472-4146","type":"electronic"}],"subject":[],"published":{"date-parts":[[2007,11,27]]}}}