Increased incidence of adult T cell leukemia-lymphoma and peripheral T cell lymphoma—not otherwise specified with limited improvement in overall survival: a retrospective analysis using data from the population-based Osaka Cancer Registry

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Peripheral T cell lymphomas (PTCL) are a heterogeneous group of non-Hodgkin lymphomas with poor outcomes. Adult T cell leukemia-lymphoma (ATL) and PTCL—not otherwise specified (PTCL-NOS)—are 2 common mature T cell lymphomas in Japan. Since it is unclear whether novel agents and treatment strategies incorporating hematopoietic cell transplantation have contributed to improved clinical outcomes in the real world, we performed a retrospective analysis using data from the population-based Osaka Cancer Registry. From 1977 to 2014, 1274 and 1143 patients were diagnosed with ATL or PTCL-NOS, respectively. Recently, the incidence of both diseases has gradually increased, and the age at diagnosis has risen. The 3-year overall survival (OS) rates in ATL patients were 12.0% in era 1 (1977–1999), 12.4% in era 2 (2000–2008), and 17.5% in era 3 (2009–2014) (P < 0.001). The 3-year OS rates in PTCL-NOS patients were 27.6% in era 1, 36.2% in era 2, and 35.0% in era 3 (P = 0.049). In conclusion, the incidences of ATL and PTCL-NOS have been increasing, particularly in elderly individuals. Clinical outcomes have improved in recent decades but are still unsatisfactory in both diseases. Thus, effective new treatment strategies incorporating novel agents are needed to further improve clinical outcomes in patients with ATL and PTCL-NOS.

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This research was partially supported by the Practical Research for Innovative Cancer Control program of the Japan Agency for Medical Research and Development (17ck0106342h0001), a National Cancer Research and Development Fund (29-A-14), and Health, Labor, and Welfare Sciences Research Grants [H30-Gantaisaku-ippan-009].

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S.F., S.K., K.N., and T.M. designed the research. S.F. and K. N analyzed the data. and S.F., S.K., K.N., T.M., I.M., and J. I wrote the paper. All the authors agreed to submit this article.

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Correspondence to Shigeo Fuji.

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The authors declare that they have no conflict of interest.

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The study was approved by the institutional review board of the Osaka International Cancer Institute in Osaka, Japan (No. 19088).

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Informed consent was obtained from all individual participants included in the study.

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The original version of this article was revised: This article was originally published without the figures 1C and 1D, thus, it requested to be revised.

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Fuji, S., Kida, S., Nakata, K. et al. Increased incidence of adult T cell leukemia-lymphoma and peripheral T cell lymphoma—not otherwise specified with limited improvement in overall survival: a retrospective analysis using data from the population-based Osaka Cancer Registry. Ann Hematol 100, 157–165 (2021).

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  • Adult T cell leukemia-lymphoma
  • Peripheral T cell lymphoma
  • Hematopoietic stem cell transplantation