Adult T-Cell Leukemia/Lymphoma

Abstract

A type of peripheral T-cell lymphoma/leukemia caused by infection with human T-cell leukemia virus 1 (HTLV-1), adult T-cell leukemia/lymphoma (ATLL) usually affects adults, with a mean age of 58 years, but with a wide age range. The male to female ratio is 1.5:1. Most patients with ATLL live in areas endemic for HTLV-1 infection, including Southwestern Japan, the Caribbean basin, and parts of coastal South America and Central Africa. This distribution of disease has been hypothesized to be linked to naval commerce routes in earlier centuries, involving Africa and other countries, with the virus probably crossing into humans initially in Africa. In endemic areas, there is a long latency between HTLV-I infection and onset of ATLL. Therefore, HTLV-1 infection is thought to occur early in life. In Japan, 2.5 % of HTLV-I carriers may develop ATLL. Sporadic cases of ATLL are also reported. ATLL therefore can occur rarely in North America and Europe in the absence of a travel history to an endemic area. HTLV-1 infection is also associated with other diseases in endemic regions including uveitis, lymphadenopathy, and a syndrome known as tropical spastic paraparesis.