Adult T cell leukemia lymphoma

Adult T cell leukemia lymphoma (ATLL) is a CD4+ lymphoproliferative malignancy resulting from human T-cell leukemia virus type 1 (HTLV-1) infection. It includes differing clinical forms classified as smoldering, chronic, lymphomatous, and acute ATLL. The Tax protein of HTLV-1 has been implicated as a viral oncoprotein which enhances virus replication and alters cellular gene expression, including activation of nuclear factor kappa B (NF kB), to result in lymphoid transformation. Chemotherapy for ATLL has had limited efficacy with median survivals of about 1 yr. Antiviral therapy employing zidovudine and interferon has shown promising results, as have antibody-based therapies to the interleukin 2 (IL2) receptor. Novel approaches employ a combination of chemo/antiretroviral therapy, hematopoietic stem cell transplantation, or inhibitors of NF kB activation.