Key Points• An improvement of 4-year OS for acute and lymphoma types of ATL was observed in comparison with that of the 1991 report.• The prognosis of the smoldering type ATL was worse than expected from the 1991 report.Adult T-cell leukemia/lymphoma (ATL) is a malignancy of mature T lymphocytes caused by human T-lymphotropic virus type I. Intensive combination chemotherapy and allogeneic hematopoietic stem cell transplantation have been introduced since the previous Japanese nationwide survey was performed in the late 1980s. In this study, we delineated the current features and management of ATL in Japan. The clinical data were collected retrospectively from the medical records of patients diagnosed with ATL between 2000 and 2009, and a total of 1665 patients' records were submitted to the central office from 84 institutions in Japan. Seventy-one patients were excluded; 895, 355, 187, and 157 patients with acute, lymphoma, chronic, and smoldering types, respectively, remained. The median survival times were 8.3, 10.6, 31.5, and 55.0 months, and 4-year overall survival (OS) rates were 11%, 16%, 36%, and 52%, respectively, for acute, lymphoma, chronic, and smoldering types. The number of patients with allogeneic hematopoietic stem cell transplantation was 227, and their median survival time and OS at 4 years after allogeneic hematopoietic stem cell transplantation was 5.9 months and 26%, respectively. This study revealed that the prognoses of the patients with acute and lymphoma types were still unsatisfactory, despite the recent progress in treatment modalities, but an improvement of 4-year OS was observed in comparison with the previous survey. Of note, one-quarter of patients who could undergo transplantation experienced long survival. It is also noted that the prognosis of the smoldering type was worse than expected. (Blood. 2015;126(24):2570-2577 Introduction Adult T-cell leukemia/lymphoma (ATL) is a peripheral T-cell malignancy caused by human T-cell lymphotropic virus type I (HTLV-1).
1-3Southwestern Japan is one of the most endemic areas for the malignancy, along with the Caribbean basin, Central and South America, and Western Africa. The Japan Clinical Oncology Group-Lymphoma Study Group proposed 4 clinical subtypes, namely, acute, lymphoma, chronic, and smoldering, based on nationwide surveys of patients with ATL who were newly diagnosed from 1983 to 1987 (1991 database). 4 Moreover, patients with the chronic type are further divided into 2 categories by the presence of any unfavorable prognostic factors, defined by levels of blood urea nitrogen (BUN) or lactate dehydrogenase (LDH) at higher than the upper limit of normal or having albumin levels lower than the lower limit of normal. Patients with acute, lymphoma, and chronic type with unfavorable prognostic factors, and those with chronic type without unfavorable prognostic factors and with smoldering type, are categorized as having aggressive and indolent There is an Inside Blood Commentary on this article in this issue.The publication costs of t...
