Division of Hematology Edirne, Department of Internal Medicine, Trakya University School of Medicine, Balkan Campus, 22030, Edirne, Turkey.
Division of Hematology, Department of Internal Medicine, Uludag University School of Medicine, Bursa, Turkey.
Ann Hematol. 2022 May;101(5):1049-1057. doi: 10.1007/s00277-022-04798-8. Epub 2022 Feb 21.
Acute promyelocytic leukemia (APL) differs from other forms of acute myeloid leukemia (AML), including coagulopathy, hemorrhage, disseminated intravascular coagulation (DIC), and treatment success with all-trans retinoic acid (ATRA). Despite ATRA, early deaths (ED) are still common in APL. Here, we evaluated factors associated with ED and applicability of scoring systems used to diagnose DIC. Ninety-one APL patients (55 females, 36 males, and median age 40 years) were included. ED was defined as deaths attributable to any cause between day of diagnosis and following 30th day. DIC was assessed based on DIC scoring system released by the International Society of Thrombosis and Hemostasis (ISTH) and Chinese Diagnostic Scoring System (CDSS). Patients' median follow-up time was 49.2 months, and ED developed in 14 (15.4% of) cases. Patients succumbing to ED had higher levels of the Eastern Cooperative Oncology Group Performance Status (ECOG PS), lactate dehydrogenase (LDH), and ISTH DIC, and lower fibrinogen levels (p <0.05). In multivariate Cox regression analysis, age >55 and ECOG PS ≥2 rates were revealed to be associated with ED. Based on ISTH and CDSS scores, DIC was reported in 47.3 and 58.2% of the patients, respectively. Despite advances in APL, ED is still a major obstacle. Besides the prompt recognition and correction of coagulopathy, those at high ED risk are recommended to be detected rapidly. Implementation of local treatment plans and creating awareness should be achieved in hematological centers. Common utilization of ATRA and arsenic trioxide (ATO) may be beneficial to overcome ED and coagulopathy in APL patients.
急性早幼粒细胞白血病 (APL) 与其他形式的急性髓细胞白血病 (AML) 不同,包括凝血功能障碍、出血、弥漫性血管内凝血 (DIC) 和全反式维甲酸 (ATRA) 的治疗成功。尽管使用了 ATRA,但 APL 患者仍常有早期死亡 (ED)。在这里,我们评估了与 ED 相关的因素以及用于诊断 DIC 的评分系统的适用性。共纳入 91 例 APL 患者(55 例女性,36 例男性,中位年龄 40 岁)。ED 定义为从诊断之日到第 30 天以后任何原因导致的死亡。DIC 根据国际血栓和止血学会 (ISTH) 和中国诊断评分系统 (CDSS) 发布的 DIC 评分系统进行评估。患者的中位随访时间为 49.2 个月,有 14 例(15.4%)发生 ED。ED 死亡患者的东部合作肿瘤学组表现状态 (ECOG PS)、乳酸脱氢酶 (LDH)、ISTH DIC 评分较高,纤维蛋白原水平较低(p<0.05)。多变量 Cox 回归分析显示,年龄>55 岁和 ECOG PS≥2 与 ED 相关。根据 ISTH 和 CDSS 评分,分别有 47.3%和 58.2%的患者发生 DIC。尽管 APL 取得了进展,但 ED 仍然是一个主要障碍。除了及时识别和纠正凝血功能障碍外,还建议快速检测那些 ED 风险较高的患者。应在血液学中心实现当地治疗计划的实施和意识的提高。在 APL 患者中,常规使用 ATRA 和三氧化二砷 (ATO) 可能有助于克服 ED 和凝血功能障碍。