Alta frequência de fatores de alto risco e de mortalidade precoce em pacientes brasileiros com leucemia promielocítica aguda

O tratamento da leucemia promielocitica aguda (LPA) com antrciclinicos e acido trans-retinoico (ATRA) tem sido amplamente empregado e resultou em taxas de sobrevida a longo prazo de 80% a 90% em diferentes ensaios clinicos. A despeito da alta prevalencia de LPA na America Latina, a efetividade de regimes de tratamento com ATRA e antraciclinicos nao e conhecida. No Brasil, mais de 20% das leucemias mieloides agudas sao do subtipo LPA. Neste estudo descrevemos uma analise retrospectiva de 157 pacientes brasileiros com LPA. Comparado com pacientes de paises desenvolvidos, observamos uma alta prevalencia de pacientes de alto risco e ma sobrevida e tres anos de 49,9%. A taxa de mortalidade precoce foi de 28%, principalmente devido a sangramento (88,6%), com 45,2% dos pacientes apresentando evidencias laboratoriais de coagulacao intravascular disseminada ao diagnostio. A despeito do fato de que no foram excluidos pacientes com base na idade ou no performance status, esta alta taxa de obito mostra que e necessaria uma melhora urgente no acesso dos pacientes a centros medicos especializados. Therapy based on anthracyclines and all-trans-retinoic acid (ATRA) has been widely used for acute promyelocytic leukemia (APL) and result in long term survival rates of 80% to 90% in different clinical trials. Despite the higher incidence of APL in Latin America, the effectiveness of ATRA + anthracyclines treatment is not known. In Brazil, more than 20% of acute myeloid leukemia are of the APL subtype. We describe a retrospective analysis including 157 Brazilian APL patients. Compared to developed countries, a higher incidence of higher incidence of high risk patients was observed and the overwall survival in three years was only 49.9%. Early mortality was 28%, mainly due to bleeding (88.6%), and laboratorial evidence of disseminated intravascular coagulation at diagnosis was present in 45.2% of the patients. Despite the fact that no patient was excluded based on age and performance status, the high death rates shows that urgent improvement in acess to specialized medical care is necessary in Brazil. Aiming to improve the outcome of APL patients in developing countries, the American Society of Hematology launched the International Consortium on APL, an educational iniative based on the use of an unified simplified treatment protocol, on line discussion tools and centralized laboratory diagnosis.