de Figueiredo-Pontes Lorena Lobo, Catto Luiz Fernando Bazzo, Chauffaille Maria de Lourdes Lopes Ferrari, Pagnano Katia Borgia Barbosa, Madeira Maria Isabel Ayrosa, Nunes Elenaide Coutinho, Hamerschlak Nelson, de Andrade Silva Marcela Cavalcante, Carneiro Thiago Xavier, Bortolheiro Teresa Cristina, de Freitas Tiago Thalles, Bittencourt Rosane Isabel, Maranhão Fagundes Evandro, Magalhães Rego Eduardo
Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto, Universidade de São Paulo, Ribeirão Preto, SP, Brazil.
Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto, Universidade de São Paulo, Ribeirão Preto, SP, Brazil; Translational Stem Cell Biology Branch, National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, MD, USA.
Hematol Transfus Cell Ther. 2024 Oct-Dec;46(4):553-569. doi: 10.1016/j.htct.2024.05.002. Epub 2024 May 18.
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.
自2013年上次发布关于急性早幼粒细胞白血病诊断和治疗的建议以来，临床评估已有所改进。在此，巴西血液学、血液治疗和细胞治疗协会的一个专家委员会对当前知识进行了全面综述，重点关注诊断、风险评估以及一线和挽救治疗方面的进展。探讨了紧急诊断的概念以及诸如凝血病和分化综合征等危急情况的管理。介绍了仅基于白细胞计数的风险分层的最新调整，以及针对非高危患者采用无化疗方案的情况。讨论了儿童、老年人和孕妇等特殊情况下的急性早幼粒细胞白血病。最后，急性早幼粒细胞白血病被视为一种高度可治愈的疾病，因为确实有可能进行针对分化的靶向治疗，而矛盾的是，它也是一种严重且紧急的疾病，值得迅速识别和处理以避免早期死亡。
