Adult T-cell leukemia-lymphoma (ATL) and its causative agent, human T-cell leukemia virus type 1 (HTLV-1), are endemic in Southwestern Japan, the Caribbean islands, Central/South America, and Intertropical Africa. Nearly ten million people worldwide are infected with HTLV-1. HTLV-1 can be transmitted via breastfeeding from mother to child, sexual intercourse, and blood transfusions. ATL occurs mostly in those infected via breastfeeding, but rarely in those infected after adolescence. Male HTLV-1 carriers have a threefold higher risk of developing ATL than female carriers. The average age at onset of ATL is late 60s in Japan, whereas it is around 40s in Central/South America. The estimated lifetime risk of developing ATL in HTLV-1 carriers is 6–7% for men and 2–3% for women in Japan. A lower anti-Tax reactivity, higher levels of soluble interleukin-2 receptor and soluble CD30, higher anti-HTLV-1 titer, and a higher proviral load (>4 copies/100 peripheral blood mononuclear cells) are known risk markers for progression of ATL. Host susceptibility, including individuals who are co-infected with Strongyloides, or those receiving immunosuppressive treatment, may be related to the development of ATL. Several promising vaccines and antibodies are under investigation to eliminate or neutralize HTLV-1 infection or to prevent the development of ATL.


Epidemiology Incidence Risk factor Transmission Prevention 


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Copyright information

© Springer Japan KK 2017

Authors and Affiliations

  1. 1.Department of Frontier Life ScienceNagasaki University Graduate School of Biomedical SciencesNagasakiJapan

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