ABSTRACT
Adult T-cell leukemia/lymphoma (ATLL) is a peripheral T-cell lymphoproliferative disorder associated with a retrovirus, human T-cell leukemia virus type 1 (HTLV-1) and is characterized by highly pleomorphic lymphoid cells. A borderline state between healthy carriers of HTLV-1 and ATLL has been named pre-ATLL. Pre-ATLL can resolve or progress into one of the four variants of ATLL. Indolent variants of ATLL can progress to aggressive variants, occasionally referred to as “crisis variants”. The lymphomatous type of ATLL is characterized by prominent adenopathy and often shows other features seen in leukemic form, but without blood involvement. Patients with ATLL with papules and nodules have poorer prognosis than those with erythema. The median survival time of patients with monoclonal proviral DNA integration in cutaneous lesions was 14 months, which was shorter than that of patients with negative proviral DNA. Patients with ATLL benefit from aggressive chemotherapy, antiretroviral therapy, bone marrow transplantation, and new drugs.
