Abstract
Adult T-cell leukemia-lymphoma (ATL) is a distinct peripheral T-lymphocytic malignancy associated with the human T-cell lymphotropic virus type I (HTLV-1). The diversity in clinical features and prognosis of patients with this disease has led to its subclassification into the following four categories : acute, lymphoma, chronic, and smoldering types. Patients with aggressive ATL generally have a poor prognosis despite chemotherapy or hematopoietic stem cell transplantation. The chronic and smoldering subtypes are considered indolent and are usually managed with topical treatment mainly for skin lesion and watchful waiting (WW) policy for systemic chemotherapy, until disease progression. The long-term prognosis of WW for indolent ATL was not good in a large-scale analysis from Japan. As for the treatment of indolent ATL, especially with skin involvement, a combination of zidovudine and interferon alpha was reported to be promising as compared to chemotherapy. However, this combination has not been evaluated in Japan. Several new agents against T-cell malignancies including ATL are now under investigation.[Skin Cancer (Japan) 2009 ; 24 : 199-205]
