Nosaka Kisato, Iwanaga Masako, Imaizumi Yoshitaka, Ishitsuka Kenji, Ishizawa Kenichi, Ishida Yoji, Amano Masahiro, Ishida Takashi, Uike Naokuni, Utsunomiya Atae, Ohshima Koichi, Kawai Kazuhiro, Tanaka Junji, Tokura Yoshiki, Tobinai Kensei, Watanabe Toshiki, Uchimaru Kaoru, Tsukasaki Kunihiro
Department of Hematology, Kumamoto University School of Medicine, Kumamoto, Japan.
Department of Frontier Life Science, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan.
Cancer Sci. 2017 Dec;108(12):2478-2486. doi: 10.1111/cas.13398. Epub 2017 Oct 8.
Adult T-cell leukemia-lymphoma (ATL) is a mature T-cell malignancy associated with human T-cell leukemia virus type 1 (HTLV-1) infection. Japan is the most endemic country for HTLV-1 and ATL in the world. Recent nationwide studies of Japanese blood donors reported that HTLV-1 carriers spread from endemic areas to non-endemic areas. Therefore, the latest information on nationwide epidemiological and clinical data for ATL is necessary to guide clinical practice. We undertook a multicenter, hospital-based survey of newly diagnosed ATL patients from 2010 to 2011. A total of 996 patients with ATL were registered from 126 hospitals across Japan. Of those, 922 (487 men and 435 women) were included in the analysis. The median age at diagnosis was 68 years (interquartile range, 60-75 years). Overall, 67.2% of ATL was diagnosed in the Kyushu-Okinawa area. The most common subtype was acute (49.5%), followed by lymphoma (25.7%), chronic (14.2%), and smoldering (10.6%). Lymphoma type was more prevalent in men (60%), whereas chronic was more prevalent in women (60%). Half of patients with lymphoma type were aged over 70 years, whereas one-third of patients with the chronic type were aged under 60 years. All of these characteristics were different from those of the previous nationwide surveys in the 1980s and 1990s. This survey clarified that half of current patients with ATL are aged over 68 years who were unable to receive intensive cytotoxic therapies. New less toxic agents for aged patients and further strategies to prevent the development of ATL from HTLV-1 carrier status are needed.
成人T细胞白血病-淋巴瘤（ATL）是一种与1型人类T细胞白血病病毒（HTLV-1）感染相关的成熟T细胞恶性肿瘤。日本是世界上HTLV-1和ATL流行最为严重的国家。近期针对日本献血者的全国性研究报告称，HTLV-1携带者正从流行地区扩散至非流行地区。因此，有必要掌握关于ATL全国流行病学和临床数据的最新信息，以指导临床实践。我们在2010年至2011年期间对新诊断的ATL患者进行了一项基于医院的多中心调查。日本全国126家医院共登记了996例ATL患者。其中，922例（487名男性和435名女性）纳入分析。诊断时的中位年龄为68岁（四分位间距为60 - 75岁）。总体而言，67.2%的ATL病例在九州-冲绳地区被诊断出来。最常见的亚型是急性型（49.5%），其次是淋巴瘤型（25.7%）、慢性型（14.2%）和冒烟型（10.6%）。淋巴瘤型在男性中更为常见（60%），而慢性型在女性中更为常见（60%）。淋巴瘤型患者中有一半年龄超过70岁，而慢性型患者中有三分之一年龄在60岁以下。所有这些特征均与20世纪80年代和9年代的上一次全国性调查结果不同。这项调查表明，目前一半的ATL患者年龄超过68岁，无法接受强化细胞毒性疗法。需要为老年患者研发毒性更低的新型药物，并制定进一步的策略来预防HTLV-1携带者发展为ATL。
