Adult T-cell leukaemia (ATL) was first reported in Japan, where it has a high incidence in the southwest region. The retrovirus human T-lymphotropic virus type I (HTLV-I) is the cause of ATL; and in ATL-endemic areas, the rate of carriage of antibodies to HTLV-I is high. A definite diagnosis of ATL is based on the presence of HTLV-I proviral DNA in the tumour-cell DNA. ATL cells originate from the CD4 subset of peripheral T cells.
ATL shows diverse clinical features but can be divided into four subtypes-acute, chronic, smouldering, and lymphoma type. It is resistant to chemotherapy, and the acute and lymphoma types have a poor prognosis. Familial occurrence of ATL is common. HTLV-I infection is caused by transmission of live infected lymphocytes from mother to child, from man to woman, or by transfusion.
Infection with HTLV-I can lead to other diseases, including HTLV-I-associated myelopathy/tropical spastic paraparesis and HTLV-I uveitis, possibly via induction of immunodeficiency or hyperreactivity against HTLV-I-infected cells.