Skip to main content
Log in

Characterization of patients with aggressive adult T-cell leukemia–lymphoma in Okinawa, Japan: a retrospective analysis of a large cohort

  • Original Article
  • Published:
International Journal of Hematology Aims and scope Submit manuscript

Abstract

Okinawa Prefecture, located in the subtropics, is an area of endemic adult T-cell leukemia–lymphoma (ATL) in Japan. We retrospectively analyzed 659 patients with aggressive ATL in seven institutions in Okinawa between 2002 and 2011. The median patient age was 68 years. More patients were aged ≥90 years (2.6 %), in this study, than in a nationwide survey (<1 %). The median survival time (MST) of the entire cohort was 6.5 months. Of the 217 patients who had a clinical status similar to that stated in the eligibility criteria of JCOG9801 (a randomized phase III study comparing VCAP-AMP-VECP with CHOP-14), 147 who received the CHOP regimen had a poorer MST than those in the CHOP-14 arm of JCOG9801 (8 vs 11 months). The prevalence of strongyloidiasis in the ATL patients was much higher (12.4 %) than in the historical cohort who visited the University of the Ryukyus Hospital (3.4 %). Furthermore, strongyloidiasis may be associated with ATL-related deaths. These findings suggest that, compared with other areas in Japan, in Okinawa, the proportion of patients aged ≥90 years with clinical features of aggressive ATL is higher, outcomes are poorer, and the disease is associated with a higher prevalence of strongyloidiasis.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5

Similar content being viewed by others

References

  1. Uchiyama T, Yodoi J, Sagawa K, Takatsuki K, Uchino H. Adult T-cell leukemia: clinical and hematologic featuresof 16 cases. Blood. 1977;50:481–92.

    CAS  PubMed  Google Scholar 

  2. Poiesz BJ, Ruscetti FW, Gazdar AF, Bunn PA, Minna JD, Gallo RC. Detection and isolation of type C retrovirus particles from fresh and cultured lymphocytes of a patient with cutaneous T-cell lymphoma. Proc Natl Acad Sci USA. 1980;77:7415–9.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  3. Hinuma Y, Nagata K, Hanaoka M, Nakai M, Matsumoto T, Kinoshita KI, et al. Adult T-cell leukemia: antigen in an ATL cell line and detection of antibodies to the antigen in human sera. Proc Natl Acad Sci USA. 1981;78:6476–80.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  4. Miyoshi I, Kubonishi I, Yoshimoto S, Akagi T, Ohtsuki Y, Shiraishi Y, et al. Type C virus particles in a cord T-cell line derived by co-cultivating normal cord leukocytes and human leukaemic T cells. Nature. 1981;294:770–1.

    Article  CAS  PubMed  Google Scholar 

  5. Yoshida M, Miyoshi I, Hinuma Y. Isolation and characterization of retrovirus from cell lines of human adult T cell leukemia and its implication in the disease. Proc Natl Acad Sci USA. 1982;79:2031–5.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  6. Shimoyama M. Diagnostic criteria and classification of clinical subtypes of adult T-cell leukaemia-lymphoma: a report from the Lymphoma Study Group (1984–87). Br J Haematol. 1991;79:428–37.

    Article  CAS  PubMed  Google Scholar 

  7. Shimoyama M. Chemotherpy of ATL. In: Takatsuki K, editor. Adult T-cell leukemia. United Kingdom: Oxford University Press, Oxford; 1994. p. 221–37.

    Google Scholar 

  8. Tobinai K, Watanabe T. Adult T-cell leukemia–lymphoma. In: Abeloff MD, Armitage JO, Niederhuber JE, Kastan MCB, editors. Clinical oncology, 3rd ed. Philadelphia: Elsevier Churchill Livingstone; 2004. p. 3109–30.

  9. Shimoyama M, Ota K, Kikuchi M, Yunoki K, Konda S, Takatsuki K, et al. Chemotherapeutic results and prognostic factors of patients with advanced non-Hodgkin’s lymphoma treated with VEPA or VEPA-M. J Clin Oncol. 1988;6:128–41.

    CAS  PubMed  Google Scholar 

  10. Lymphoma Study Group. Major prognostic factors of patients with adult T-cell leukemia–lymphoma: a cooperative study—Lymphoma Study Group (1984–1987). Leuk Res. 1991;15:81–90.

    Article  Google Scholar 

  11. Tobinai K, Shimoyama M, Minato K, Shirakawa S, Kobayashi T, Hotta T. Japan Clinical Oncology Group phase II trial of second-generation “LSG4 protocol” in aggressive T- and B-lymphoma: a new predictive model for T- and B-lymphoma. Proc Am Soc Clin Oncol. 1994;13:378.

    Google Scholar 

  12. Tsukasaki K, Tobinai K, Shimoyama M, Kozuru M, Uike N, Yamada Y, et al. Lymphoma Study Group of the Japan Clinical Oncology Group. Deoxycoformycin-containing combination chemotherapy for adult T-cell leukemia–lymphoma: Japan Clinical Oncology Group Study (JCOG9109). Int J Hematol. 2003;77:164–70.

    Article  CAS  PubMed  Google Scholar 

  13. Yamada Y, Tomonaga M, Fukuda H, Hanada S, Utsunomiya A, Tara M, et al. A new G-CSF supported combination chemotherapy, LSG15, for adult T-cell leukaemia-lymphoma: Japan Clinical Oncology Group Study 9303. Br J Haematol. 2001;113:375–82.

    Article  CAS  PubMed  Google Scholar 

  14. Tsukasaki K, Utsunomiya A, Fukuda H, Shibata T, Fukushima T, Takatsuka Y, Japan Clinical Oncology Group Study JCOG9801, et al. VCAP-AMP-VECP compared with biweekly CHOP for adult T-cell leukemia–lymphoma: Japan Clinical Oncology Group Study JCOG9801. J Clin Oncol. 2007;25:5458–64.

    Article  CAS  PubMed  Google Scholar 

  15. Hishizawa M, Kanda J, Utsunomiya A, Taniguchi S, Eto T, Moriuchi Y, et al. Transplantation of allogeneic hematopoietic stem cells for adult T-cell leukemia: a nationwide retrospective study. Blood. 2010;116:1369–76.

    Article  CAS  PubMed  Google Scholar 

  16. Ishida T, Hishizawa M, Kato K, Tanosaki R, Fukuda T, Taniguchi S, et al. Allogeneic hematopoietic stem cell transplantation for adult T-cell leukemia–lymphoma with special emphasis on preconditioning regimen: a nationwide retrospective study. Blood. 2012;120:1734–41.

    Article  CAS  PubMed  Google Scholar 

  17. Kanda J, Hishizawa M, Utsunomiya A, Taniguchi S, Eto T, Moriuchi Y, et al. Impact of graft-versus-host disease on outcome after allogeneic hematopoietic cell transplantation for adult T-cell leukemia: a retrospective cohort study. Blood. 2012;119:2141–8.

    Article  CAS  PubMed  Google Scholar 

  18. Tajima K, The T- and B-cell Malignancy Study Group. The 4th nationwide study of adult T-cell leukemia/lymphoma (ATL in Japan): estimates of risk of ATL and its geographical and clinical features. Int J Cancer. 1990;45:237–43.

    Article  CAS  PubMed  Google Scholar 

  19. Yamada Y, Atogami S, Hasegawa H, Kamihira S, Sohda M, Satake M, Yamaguchi K. Nationwide survey of adult T-cell leukemia/lymphoma (ATL) in Japan. Rinshoketsueki. 2011;52:1765–71.

    Google Scholar 

  20. Hirata T, Nakamura H, Kinjo N, Hokama A, Kinjo F, Yamane N, Fujita J. Prevalence of blastocystis hominis and Strongyloides stercoralis infection in Okinawa, Japan. Parasito Res. 2007;101:1717–9.

    Article  Google Scholar 

  21. Makiyama J, Imaizumi Y, Tsushima H, Taniguchi H, Moriwaki Y, Sawayama Y, et al. Treatment outcome of elderly patients with aggressive adult T-cell leukemia–lymphoma: Nagasaki University Hospital experience. Int J Hematol. 2014;100:464–72.

    Article  CAS  PubMed  Google Scholar 

  22. Ishida T, Jo T, Takemoto S, Suzushima H, Uozumi K, Yamamoto K, et al. Dose-intensified chemotherapy alone of in combination with mogamulizumab in newly diagnosed aggressive adult T-cell leukaemia–lymphoma: a randomized phase II study. Br J Haematol. 2015;169:672–82.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  23. O’Doherty MJ, Van de Pette JE, Numan TO. Recurrent Strongyloides stercoralis infection in a patient with T-cell lymphoma–leukemia (letter). Lancet. 1984;1(8381):858.

    Article  PubMed  Google Scholar 

  24. Nakada K, Yamaguchi K, Furugen S, Nakasone T, Oshiro Y, Hinuma Y, et al. Monoclonal integration of HTLV-1 proviral DNA in patients with strongyloidiasis. Int J Cancer. 1987;40:145–8.

    Article  CAS  PubMed  Google Scholar 

  25. Gotuzzo E, Terashima A, Alvarez H, Tello R, Infante R, Watts DM, Freedman DO. Strongyloides stercoralis hyperinfection associated with human T-cell lymphotropic virus type-I infection in Peru. Am J Trop Med Hyg. 1999;60:146–9.

    CAS  PubMed  Google Scholar 

  26. Newton RC, Limpuangthip P, Greenberg S, Gam A, Neva FA. Strongyloides stercoralis hyperinfection in a carrier of HTLV-1 virus with evidence of selective immunosuppression. Am J Med. 1992;92:202–8.

    Article  CAS  PubMed  Google Scholar 

  27. Phelps KR. Strongyloides hyperinfection in patients coinfected with HTLV-1 and S. stercoralis. Am J Med. 1993;94:447–9.

    Article  CAS  PubMed  Google Scholar 

Download references

Acknowledgments

This work was supported by a grant from Okinawa Prefecture, Japan (grant to T.F. and T.T.).

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Takuya Fukushima.

Ethics declarations

Conflict of interest

The authors report no potential conflict of interest.

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Nishi, Y., Fukushima, T., Nomura, S. et al. Characterization of patients with aggressive adult T-cell leukemia–lymphoma in Okinawa, Japan: a retrospective analysis of a large cohort. Int J Hematol 104, 468–475 (2016). https://doi.org/10.1007/s12185-016-2042-y

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12185-016-2042-y

Keywords

Navigation