Encyclopedia of Pathology

Living Edition
| Editors: J.H.J.M. van Krieken

Adult T-Cell Leukemia/Lymphoma

  • Atsuko NakazawaEmail author
  • Koichi Ohshima
Living reference work entry
DOI: https://doi.org/10.1007/978-3-319-28845-1_3865-1


Adult T-cell leukemia/lymphoma (ATLL) is a peripheral T-cell neoplasm most often composed of highly pleomorphic lymphoid cells. The disease is usually widely disseminated and is caused by the human retrovirus known as human T-cell leukemia virus type 1 (HTLV-1)(Ohshima et al. 2017).

Clinical Features

  • Incidence

ATLL is endemic in several regions of the world: southwestern Japan, the Caribbean basin, and parts of Central Africa. Other areas of prevalence include Central and South America, in particular Brazil and Ecuador. The cumulative incidence of ATLL is estimated to be 2.5% among HTLV-1 carriers in Japan, with an ongoing increased risk until 70 years of age (Tajima 1999). ATLL occurs only in adults. Differences in geographic or ethnic origin correlate with the different patterns of disease. A lymphomatous form is more often observed in the Western hemisphere, while Asian patients are more often leukemic (Levine et al. 1994).
  • Age

The disease has a long latency, and affected...
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References and Further Reading

  1. Bunn, P. A., Jr., Schechter, G. P., Jaffe, E., et al. (1983). Clinical course of retrovirus-associated adult T-cell lymphoma in the United States. The New England Journal of Medicine, 309(5), 257–264.CrossRefGoogle Scholar
  2. Fukushima, T., Miyazaki, Y., Honda, S., et al. (2005). Allogeneic hematopoietic stem cell transplantation provides sustained long-term survival for patients with adult T-cell leukemia/lymphoma. Leukemia, 19(5), 829–834.CrossRefGoogle Scholar
  3. Itoyama, A., Chaganti, R. S. K., Yamada, Y., et al. (2001). Cytogenetic analysis and clinical significance in adult T-cell leukemia/lymphoma: A study of 50 cases from the human T-cell leukemia virus type-1 endemic area, Nagasaki. Blood, 97(11), 3612–3620.CrossRefGoogle Scholar
  4. Jaffe, E. S., Blattner, W. A., Blayney, D. W., et al. (1984). The pathologic spectrum of adult T-cell leukemia/lymphoma in the United States. The American Journal of Surgical Pathology, 8, 263–275.CrossRefGoogle Scholar
  5. Levine, P. H., Manns, A., Jaffe, E. S., et al. (1994). The effect of ethnic differences on the pattern of HTLV-I-associated T-cell leukemia/lymphoma (HATL) in the United States. International Journal of Cancer, 56(2), 177–181.CrossRefGoogle Scholar
  6. Ohshima, K., Suzumiya, J., Sato, K., et al. (1999). Survival of patients with HTLV-I-associated lymph node lesions. The Journal of Pathology, 189(4), 539–545.CrossRefGoogle Scholar
  7. Ohshima, K., Jaffe, E.S., & Yoshino, T. (2017). Adult T-cell leukaemia/lymphoma. In S. H. Swerdlow, E. Campo, N. L. Harris, et al. (Eds.), WHO classification of tumours of haematopoietic and lymphoid tissues (Rev. 4th ed., pp. 363–367). Lyon: IARC.Google Scholar
  8. Shimoyama, M. (1999). Diagnostic criteria and classification of clinical subtypes of adult T-cell leukaemia-lymphoma. A report from the Lymphoma Study Group (1984–87). British Journal of Haematology, 79(3), 428–437.CrossRefGoogle Scholar
  9. Tajima, K. (1999). The 4th nation-wide study of adult T-cell leukemia/lymphoma (ATL) in Japan: Estimates of risk of ATL and its geographical and clinical features. The T- and B-cell Malignancy Study Group. International Journal of Cancer, 45(2), 237–243.CrossRefGoogle Scholar
  10. Takasaki, Y., Iwanaga, M., Imaizumi, Y., et al. (2010). Long-term study of indolent adult T-cell leukemia-lymphoma. Blood, 115(22), 4337–4343.CrossRefGoogle Scholar
  11. Tsukasaki, K., et al. (2007). VCAP-AMP-VECP compared with biweekly CHOP for adult T-cell leukemia-lymphoma: Japan Clinical Oncology Group Study JCOG9801. Journal of Clinical Oncology, 25(34), 5458–5464.CrossRefGoogle Scholar
  12. Tsukasaki, K., Hermine, O., Bazarbachi, A., et al. (2009). Definition, prognostic factors, treatment, and response criteria of adult T-cell leukemia-lymphoma: A proposal from an international consensus meeting. Journal of Clinical Oncology, 27(3), 453–459.CrossRefGoogle Scholar
  13. Yamaguchi, T., Ohshima, K., Karube, K., et al. (2005). Clinicopathological features of cutaneous lesions of adult T-cell leukaemia/lymphoma. The British Journal of Dermatology, 152(1), 76–81.CrossRefGoogle Scholar

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Authors and Affiliations

  1. 1.Department of Clinical ResearchSaitama Children’s Medical centerSaitamaJapan
  2. 2.Department of Pathology, School of MedicineKurume UniversityKurumeJapan