{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T19:43:17Z","timestamp":1790797397177,"version":"4.1.0"},"reference-count":29,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2020,10,21]],"date-time":"2020-10-21T00:00:00Z","timestamp":1603238400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Cancer Science"],"published-print":{"date-parts":[[2020,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Adult T\u2010cell leukemia\/lymphoma (ATL) is a mature T\u2010cell neoplasm and is classified into four subtypes (acute, lymphoma, chronic, and smoldering) according to the Shimoyama classification, established in 1991 through several nationwide surveys based on the clinical diversity of patients diagnosed in 1983\u20101987 in Japan. Thereafter, no such studies have been conducted. Recently, we conducted a nationwide hospital survey using the method of the 1980s studies, collected baseline data on 996 ATL patients diagnosed in 2010\u20102011 from 126 hospitals, and reported their unique epidemiological characteristics. Here, we report the follow\u2010up results of registered ATL patients with the goal of evaluating current prognoses and treatment modalities as of 2016\u20102017. Of 770 evaluable patients, 391 (50.8%) had acute\u2010type, 192 (24.9%) had lymphoma\u2010type, 106 (13.8%) had chronic\u2010type, and 81 (10.5%) had smoldering\u2010type ATL. The initial therapy regimens used for acute\/lymphoma\u2010type ATL were vincristine, cyclophosphamide, doxorubicin and prednisone, followed by doxorubicin, ranimustine, and prednisone and then by vindesine, etoposide, carboplatin, and prednisone (VCAP\u2010AMP\u2010VECP)\u2010like in 38.5\/41.7% and cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP)\u2010like in 14.6\/13.7% of patients. Allogeneic hematopoietic stem cell transplantation was used to treat 15.9\/10.4% of acute\/lymphoma\u2010type ATL patients. The 4\u2010year survival rates (the median survival time, days) for acute\u2010, lymphoma\u2010, unfavorable chronic\u2010, favorable chronic\u2010, and smoldering\u2010type ATL were 16.8% (252), 19.6% (305), 26.6% (572), 62.1% (1937), and 59.8% (1851), respectively. The 4\u2010year survival rates for acute\u2010 and lymphoma\u2010type ATL improved compared with those reported in 1991, but those for chronic\u2010 and smoldering\u2010type ATL were not. Further efforts are warranted to develop more efficient therapeutic strategies to improve the prognosis of ATL in Japan.<\/jats:p>","DOI":"10.1111\/cas.14658","type":"journal-article","created":{"date-parts":[[2020,9,25]],"date-time":"2020-09-25T18:24:55Z","timestamp":1601058295000},"page":"4567-4580","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":88,"title":["Prognosis of patients with adult T\u2010cell leukemia\/lymphoma in Japan: A nationwide hospital\u2010based study"],"prefix":"10.1111","volume":"111","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2954-5691","authenticated-orcid":false,"given":"Yoshitaka","family":"Imaizumi","sequence":"first","affiliation":[{"name":"Department of Hematology Nagasaki University Hospital Nagasaki Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1148-6192","authenticated-orcid":false,"given":"Masako","family":"Iwanaga","sequence":"additional","affiliation":[{"name":"Department of Clinical Epidemiology Nagasaki University School of Medicine Nagasaki Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6682-2451","authenticated-orcid":false,"given":"Kisato","family":"Nosaka","sequence":"additional","affiliation":[{"name":"Department of Hematology Kumamoto University School of Medicine Kumamoto Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7030-497X","authenticated-orcid":false,"given":"Kenji","family":"Ishitsuka","sequence":"additional","affiliation":[{"name":"Department of Hematology and Rheumatology Kagoshima University Hospital Kagoshima 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Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Naokuni","family":"Uike","sequence":"additional","affiliation":[{"name":"Department of Hospice care St. Mary\u2019s hospital Kurume Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Atae","family":"Utsunomiya","sequence":"additional","affiliation":[{"name":"Department of Hematology Imamura General Hospital Kagoshima Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Koichi","family":"Ohshima","sequence":"additional","affiliation":[{"name":"Department of Pathology Kurume University School of Medicine Kurume Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Junji","family":"Tanaka","sequence":"additional","affiliation":[{"name":"Department of Hematology Tokyo Woman\u2019s Medical University Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yoshiki","family":"Tokura","sequence":"additional","affiliation":[{"name":"Department of Dermatology Hamamatsu University School of Medicine Hamamatsu Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kensei","family":"Tobinai","sequence":"additional","affiliation":[{"name":"Department of Hematology National Cancer Center Hospital Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Toshiki","family":"Watanabe","sequence":"additional","affiliation":[{"name":"Department of Practical Management of Medical Information St. Marianna University Graduate School of Medicine Kawasaki Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kaoru","family":"Uchimaru","sequence":"additional","affiliation":[{"name":"Department of Computational Biology and Medical Sciences Graduate School of Frontier Sciences The University of Tokyo Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kunihiro","family":"Tsukasaki","sequence":"additional","affiliation":[{"name":"Department of Hematology International Medical Center Saitama Medical University Saitama Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"for collaborative Investigators","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2020,10,21]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V50.3.481.481"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.77.12.7415"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.79.6.2031"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.3389\/fmicb.2012.00388"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/S2214-109X(16)30143-7"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/jmv.23181"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1473-3099(16)30252-3"},{"key":"e_1_2_8_9_1","first-page":"322","article-title":"Adult 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