Adult T cell leukemia-lymphoma in Brasil: epidemiology, treatment and controverstial aspects

Abstract

Adult T-cell leukemia/lymphoma (ATLL) is a malignant disease of T-cell mature lymphocyte associated to human T-cell lymphotropic virus type I (HTLV-I). Although ATLL frequently occurs in regions where HTLV-I infection are endemic, such as Japan and Caribbean islands, however sporadic cases have been described in HTLV-I non endemic areas. In Brazil, the most recognized clinical form of ATLL are acute and lymphoma subtypes. The occurrence of ATLL in children and young patients was an interesting and distinct demographic features observed in the Brazilian cohort. Rio de Janeiro and Salvador cities where locations with the majority of cases registered, followed by Recife and São Paulo cities. The most aggressive clinical forms of ATLL are unresponsive to conventional chemotherapy protocols with poor rate of 5 years survival. However in cases who interferon-a and ziovudine were used as consolidation treatment the overall results demonstrated a better survival rate. Recently the alternative treatment using anti-CD25 showed promising results, even though in experimental protocols. With the increasing recognition of ATLL in Brazil, it has been necessary to apply different therapeutic protocols for this disease. In this manuscript we described the clinical and epidemiological characteristics of the Brazilian cases and a review of literature regarding therapeutic approaches of ATLL seeking new insights and management of biological modifiers and retroviral drugs.