Diagnosis and management of acute promyelocytic leukemia: Brazilian consensus guidelines 2024 on behalf of the Brazilian Association of Hematology, Hemotherapy and Cellular Therapy
Lorena Lôbo de Figueiredo-Pontes, Luiz Fernando Bazzo Catto, Maria de Lourdes Chauffaille, Kátia Bórgia Barbosa Pagnano, Maria Isabel Ayrosa Madeira, Elenaide C. Nunes, Nelson Hamerschlak, Marcela Cavalcante de Andrade Silva, Thiago Xavier Carneiro, Teresa Cristina Bortolheiro, Tiago Thalles de Freitas, Rosane Bittencourt, Evandro M. Fagundes, Eduardo Magalhães Rego
Abstract
Improvements in clinical assessment have occurred since the last published recommendations on the diagnosis and treatment of acute promyelocytic leukemia in 2013. Here, a committee of specialists of the Brazilian Association of Hematology, Hemotherapy and Cellular Therapy presents a comprehensive review on the current knowledge, focusing on the advances in diagnosis, risk assessment, and frontline and salvage therapy. The concept of urgent diagnosis is explored as well as the management of critical situations such as coagulopathy and differentiation syndrome. Recent adjustments in risk stratification based on white blood cell counts only are presented together with the incorporation of chemo-free regimens for non-high-risk patients. Special conditions such as acute promyelocytic leukemia in children, the elderly and pregnant women are discussed. Finally, acute promyelocytic leukemia is presented as a highly curable disease because of the real possibility of targeted therapy towards differentiation, and, paradoxically, as a serious and urgent condition that deserves prompt recognition and management to avoid early mortality.