Adult T-cell leukemia/lymphoma (ATLL) is a rare T-cell disorder that is etiologically linked to chronic infection with human T-cell lymphotropic virus type 1. ATLL is divided into four subtypes: acute, lymphomatous, chronic, and smoldering. The acute and lymphomatous variants are often described clinically as the aggressive types of ATLL. Treatment strategies traditionally have focused on antiviral therapy with zidovudine and interferon-alpha and combination chemotherapy. Novel therapeutic approaches include the use of monoclonal antibodies, anti-CCR4 therapy, immunomodulatory therapy, and anti-TAX vaccines. Future research must focus on multi-institutional clinical trial participation because of the rarity of this deadly hematologic malignancy.
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