Adult T-cell leukemia/lymphoma (ATLL) is a mature T-cell lymphoproliferative disorder associated with the human T lymphotropic virus (HTLV-1) infection. ATLL predominantly affects individuals within HTLV-1 endemic areas such as Japan, areas of Africa, South America, and the Caribbean. HTLV-1 preferentially infects CD4+ T-cells, and several genetic hits must occur before ATLL develops. ATLL is classically divided into four clinical variants based on manifestations of disease: acute, chronic, lymphomatous, and smouldering. As of 2019, a new subtype has been described: lymphoma type of ATL, extranodal primary cutaneous. In this review, emphasis will be taken to describe the common clinicopathologic manifestations of the disease, advances in biomarker discovery, mutational landscape and targeted therapeutic approaches to treat this highly aggressive and frequently lethal type of T-cell lymphoma.
ASJC Scopus subject areas
- Pathology and Forensic Medicine