Imaizumi Yoshitaka
Department of Hematology, Nagasaki University Hospital.
Rinsho Ketsueki. 2021;62(7):760-765. doi: 10.11406/rinketsu.62.760.
Adult T-cell leukemia-lymphoma (ATL) is a rare disease, and the nationwide surveys conducted in Japan have played an important role in improving our understanding of the clinical features and prognosis of this disease. The diagnostic criteria of clinical subtypes have been proposed based on the surveys conducted on patients with ATL who were diagnosed in the 1980s; the current treatment guideline in Japan is based on this classification of ATL subtypes. In the survey for patients diagnosed between 2000 and 2009, the usefulness of the clinical subtypes was confirmed, and soluble interleukin-2 receptor was identified as a new prognostic factor for chronic- and smoldering-type ATL. We conducted another survey for patients who were diagnosed in 2010 and 2011. The age at diagnosis was higher than that reported in previous trials, and the median patient age at diagnosis was 68 years in the study. The 4-year survival rate was better than that in previous studies on acute- and lymphoma-type disease; however, the prognosis has not improved in chronic- and smoldering-type disease. Further nationwide surveys are expected to improve the treatment strategies for ATL.
成人T细胞白血病-淋巴瘤（ATL）是一种罕见疾病，日本开展的全国性调查在增进我们对该疾病临床特征和预后的了解方面发挥了重要作用。临床亚型的诊断标准是基于对20世纪80年代确诊的ATL患者进行的调查提出的；日本目前的治疗指南基于这种ATL亚型分类。在对2000年至2009年确诊患者的调查中，证实了临床亚型的实用性，并确定可溶性白细胞介素-2受体是慢性和隐匿型ATL的新预后因素。我们对2010年和2011年确诊的患者进行了另一项调查。诊断时的年龄高于以往试验报告的年龄，研究中患者诊断时的中位年龄为68岁。4年生存率优于以往关于急性和淋巴瘤型疾病的研究；然而，慢性和隐匿型疾病的预后并未改善。预计进一步的全国性调查将改善ATL的治疗策略。
