Abstract

ABSTRACT Introduction ATL is a malignancy of mature T-lymphocytes caused by human T-lymphotropic virus type I (HTLV-1), and it is classified into 4 clinical subtypes,i.e. acute, lymphoma, chronic, and smoldering type according to the analysis of the former nationwide survey performed in late 80's. During the 2 decades after this analsysis, treatment for ATL has improved, e.g. intensive sequential combination chemotherapy and allogeneic hematopoietic stem cell transplantation (HSCT). We therefore performed a nationwide survey to know the outcome of ATL patients newly diagnosed during the last decade. Patients and methods The clinical data was collected retrospectively from the medical record of patients who were diagnosed as ATL between 2000 and 2009 in participating institutions. Approval of this study was obtained from the Ethics Committee and Institutional Review Board of Fukuoka University where the central office is located and at each participating center based on their institutional policies. Results A total of 1552 patients' survey form was submitted from 81 institutions across Japan. Fifty-four patients were excluded due to missing data, and the remaining 1498 were analyzed. The median survival time (MST) of 897 for acute type and that of 355 for lymphoma type were 8.3 and 10.6 months, and overall survival (OS) rates at 5 years were 9% and 13%, respectively. Fifty % of patients had received CHOP/CHOP-like regimen, 31 % had VCAP-AMP-VECP regimen, and single agent was used in 6% of the patients. The number of patients with allogeneic HSCT was 227 (46% from a sibling donor and 51% from an unrelated donor), a half of them were transplanted at first remission. The MST and OS at 5 years from transplantation were 5 months and 25%, respectively. The MST of 172 for chronic and that of 74 for smoldering type were 30.3 and 36.7 months, respectively. Conclusion The prognosis of acute and lymphoma type was still poor despite the recent progress in treatment modalities, and it is of disappointment that their therapeutic outcome was not remarkably improved as compared to former survey. It is also a surprise that the prognosis of smoldering type is not good as expected from the previous survey. To note, 25% of patients who underwent transplantation experienced a long survival, but it should be validated by the prospective study. Disclosure All authors have declared no conflicts of interest.

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