Steffenello-Durigon Giovanna, Bigolin Alisson, Moraes Ana Carolina Rabello de, Rudolf-Oliveira Renata Cristina, Moral Joanita Angela Gonzaga Del, Santos-Silva Maria Claudia
Universidade Federal de Santa Catarina (UFSC), Florianópolis, RS, Brazil.
Universidade Federal de Santa Catarina (UFSC), Florianópolis, RS, Brazil.
Hematol Transfus Cell Ther. 2021 Jan-Mar;43(1):21-27. doi: 10.1016/j.htct.2019.12.001. Epub 2020 Jan 23.
Acute promyelocytic leukemia is a subtype of acute myeloid leukemia, characterized by the presence of neoplastic promyelocytes, due to the reciprocal balanced translocation between chromosomes 15 and 17. Currently, with the use of agents that act directly on this molecular change, such as all-trans retinoic acid and arsenic trioxide, APL has shifted from a highly mortal to a curable disease. However, some cases are still at high risk of death, especially early death, and acquiring a better understanding of the clinical and biological factors involving APL is needed to correctly identify and treat such cases. The early suspected diagnosis and prompt initiation of the target therapy are important for better response rates. The follow-up and outcomes, using real-life data from 44 consecutive APL patients, were studied between 2001 and 2013. The overall survival rate was 82.7% and early death was 16%. Almost all patient deaths were due to severe bleeding, which was confirmed by multivariate analysis, as the most important prognostic factor leading to death. A better understanding the pathogenesis of the hemorrhagic complications in APL is needed, as well as the risk factors associated with early death in APL patients, as this has become synonymous with overall mortality.
急性早幼粒细胞白血病是急性髓系白血病的一种亚型，其特征是存在肿瘤性早幼粒细胞，这是由于15号和17号染色体之间的相互平衡易位所致。目前，随着直接作用于这种分子变化的药物的使用，如全反式维甲酸和三氧化二砷，急性早幼粒细胞白血病已从一种高致死性疾病转变为可治愈的疾病。然而，一些病例仍有很高的死亡风险，尤其是早期死亡，因此需要更好地了解涉及急性早幼粒细胞白血病的临床和生物学因素，以正确识别和治疗此类病例。早期疑似诊断和及时启动靶向治疗对于提高缓解率很重要。我们利用2001年至2013年间44例连续急性早幼粒细胞白血病患者的真实生活数据，对其随访情况和预后进行了研究。总生存率为82.7%，早期死亡率为16%。几乎所有患者的死亡都归因于严重出血，多因素分析证实这是导致死亡的最重要预后因素。需要更好地了解急性早幼粒细胞白血病出血并发症的发病机制，以及与急性早幼粒细胞白血病患者早期死亡相关的危险因素，因为这已成为总体死亡率的同义词。
