Introduction: Adult T-cell leukemia/lymphoma (ATL) is a rare disease with a unique geographic distribution. Conducting controlled randomized trials to assess the effective therapeutic strategies has therefore been a significant challenge to date.
Areas covered: This review explores the natural history and diagnostic evaluation of ATL, followed by a focused review of existing studies on the most potent individual pharmaceutical agents and combinations used in the therapy of this malignancy. Readers will acquire considerable insights about the clinical subsets, diagnosis and the most effective therapies used in various ATL types.
Expert opinion: International, multicenter, randomized clinical trials are essential to design optimal therapeutic strategies for various ATL subsets. It appears that patients with acute ATL type benefit considerably from the first-line combined antiviral therapy with zidovudine and interferon alpha, whereas patients with ATL of the lymphoma type may experience a better outcome with intensive chemotherapy. The role of therapy in smoldering and chronic disease types remains to be clarified. In addition, the results of allogeneic stem-cell transplantation in ATL appear promising, as up to 40% of patients who achieve remission and have suitable donors can now become long-term survivors. Prospective evaluation of novel effective agents and their incorporation into various therapeutic algorithms is stringently needed.