Adult T-cell leukemia/lymphoma (ATL) is amalignancy ofmature T lymphocytes caused by human T-lymphotropic virus type I. Intensive combination chemotherapy and allogeneic hematopoietic stemcell transplantation have been introduced since the previous Japanese nationwide survey was performed in the late 1980s. In this study, we delineated the current features andmanagement of ATL in Japan. The clinical data were collected retrospectively fromthemedical records of patientsdiagnosedwithATLbetween 2000 and2009, anda 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, andsmoldering types, respectively, remained. Themedian survival timeswere 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 stemcell transplantation was 227, and their median survival timeandOSat 4 years after allogeneic hematopoietic stemcell transplantationwas 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 treatmentmodalities, but an improvement of 4-year OS was observed in comparisonwith 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.
ASJC Scopus subject areas
- Cell Biology