Abstract

Adult T-cell leukemia/lymphoma (ATLL) is a peripheral T-cell neoplasm with a very poor outcome. However, several studies have shown a progress in the treatment. To evaluate the effect of the progress in the treatment of ATLL in a whole patient population, we used vital statistics data and estimated age-adjusted mortality and trends in the mortality from 1995 to 2009. Since allogeneic hematopoietic stem-cell transplantation (allo-HSCT) has been introduced as a modality with curative potential during study period, we also evaluated the association of the annual number of allo-HSCT and the trend of the mortality of ATLL. Endemic (Kyushu) and non-endemic areas (others) were evaluated separately. Significance in the trend of mortality was evaluated by joinpoint regression analysis. During the study period, a total of 14 932 patients died of ATLL in Japan, and mortality decreased significantly in both areas (annual percent change (95% confidence interval (CI)): Kyushu, −3.1% (−4.3, −1.9); others, −3.4% (−5.3, −1.5)). This decreasing trend in mortality seems to be associated with an increase in the number of allo-HSCTs (Kyushu, R-squared=0.70, P=0.003; and others, R-squared=0.55, P=0.058). This study reveals that the mortality of ATLL is now significantly decreasing in Japan and this decreasing trend might be associated with allo-HSCT.

Highlights

  • Adult T-cell leukemia/lymphoma (ATLL) is a peripheral T-cell neoplasm that is associated with infection by the human T-cell leukemia virus type I.1,2 Infection with human T-cell leukemia virus type I and cases of ATLL are endemic in several regions of the world, with the south-west area of Japan (Kyushu) being a well-known example

  • Estimated age-standardized mortalities of ATLL from 1995 to 2009 in Kyushu and others are shown as circles in Figure 1 and the exact rates with 95% confidence intervals in both areas, which are the basis of Figure 1, are summarized in Supplementary Table 1

  • Allo-HSCT in patients aged 55 years or older showed an association with the decrease in the mortality suggesting that increasing the candidate of allo-HSCT in this population may improve the outcome of ATLL

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Summary

Introduction

Adult T-cell leukemia/lymphoma (ATLL) is a peripheral T-cell neoplasm that is associated with infection by the human T-cell leukemia virus type I.1,2 Infection with human T-cell leukemia virus type I and cases of ATLL are endemic in several regions of the world, with the south-west area of Japan (Kyushu) being a well-known example. Since allo-HSCT has been introduced as a modality with curative potential during study period, we evaluated the correlation of the annual number of allo-HSCT and the trend of the mortality of ATLL.

Results
Conclusion
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