Songthawee Natsaruth, Sripornsawan Pornpun, Chavananon Shevachut, Kittivisuit Sirinthip, McNeil Edward B, Chotsampancharoen Thirachit
Division of Hematology and Oncology, Department of Pediatrics, Faculty of Medicine, Prince of Songkla University, Hat Yai, Thailand.
Division of Rheumatology, Department of Pediatrics, Faculty of Medicine, Prince of Songkla University, Hat Yai, Thailand.
Pediatr Hematol Oncol. 2024;41(2):150-162. doi: 10.1080/08880018.2023.2286970. Epub 2024 Feb 10.
Few studies have reported the survival outcomes of myeloid leukemia associated with Down syndrome (DS) in resource-limited countries. This study aimed to compare characteristics and survival outcomes of children with acute myeloid leukemia (AML) between those with and without DS in Thailand. The medical records of AML patients aged 0-15 years treated in a major tertiary center in Southern Thailand between October 1978 and December 2019 were reviewed retrospectively. The overall (OS) and event-free survivals (EFS) rates were calculated using the Kaplan-Meier method. A total of 362 AML patients were included, of which 41 (11.3%) had DS. The mean age at diagnosis of the DS patients was 2.5 ± 1.9 years and most of them (90.2%) were under the age of five. The DS patients had lower initial white blood cell counts and peripheral blasts compared to the non-DS patients. The AML-M7 subtype was more common in the DS than in the non-DS patients (80.5% vs. 9.1%, < 0.01, respectively). The 5-year OS and EFS rates of the DS patients were lower compared to the non-DS patients (12.9% vs. 20.5%, = 0.05 and 13.7% vs. 18.4%, = 0.03, respectively). DS patients had a significantly higher rate of early and treatment-related deaths compared to non-DS patients (30.3% vs. 13.5%, < 0.01 and 39.4% vs. 19.5%, = 0.02, respectively). Over the study period, there were a decrease in early death rate and an increase in survival rates of DS patients, which suggests that chemotherapy regimens and supportive care have improved over time.
在资源有限的国家，很少有研究报告与唐氏综合征（DS）相关的髓系白血病的生存结果。本研究旨在比较泰国患有和未患有DS的急性髓系白血病（AML）儿童的特征和生存结果。回顾性分析了1978年10月至2019年12月在泰国南部一家主要三级中心接受治疗的0至15岁AML患者的病历。采用Kaplan-Meier方法计算总生存率（OS）和无事件生存率（EFS）。共纳入362例AML患者，其中41例（11.3%）患有DS。DS患者的诊断平均年龄为2.5±1.9岁，其中大多数（90.2%）年龄在5岁以下。与非DS患者相比，DS患者的初始白细胞计数和外周血原始细胞较低。AML-M7亚型在DS患者中比在非DS患者中更常见（分别为80.5%对9.1%，P<0.01）。与非DS患者相比，DS患者的5年OS和EFS率较低（分别为12.9%对20.5%，P = 0.05和13.7%对18.4%，P = 0.03）。与非DS患者相比，DS患者的早期和治疗相关死亡率显著更高（分别为30.3%对13.5%，P<0.01和39.4%对19.5%，P = 0.02）。在研究期间，DS患者的早期死亡率下降，生存率上升，这表明化疗方案和支持治疗随着时间的推移有所改善。
