Natsaruth Songthawee, Pornpun Sripornsawan, Shevachut Chavananon, Sirinthip Kittivisuit, Edward B McNeil, Thirachit Chotsampancharoen
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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%, <i>p</i> < 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%, <i>p</i> = 0.05 and 13.7% vs. 18.4%, <i>p</i> = 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%, <i>p</i> < 0.01 and 39.4% vs. 19.5%, <i>p</i> = 0.02, respectively). 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引用次数: 0
摘要
在资源有限的国家,很少有研究报道与唐氏综合征(DS)相关的髓系白血病的生存结局。本研究旨在比较泰国患有和不患有DS的急性髓性白血病(AML)儿童的特征和生存结局。回顾性回顾了1978年10月至2019年12月在泰国南部一家主要三级医疗中心治疗的0-15岁AML患者的医疗记录。采用Kaplan-Meier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患者的平均诊断年龄为2.5±1.9岁,以5岁以下患者居多(90.2%)。与非DS患者相比,DS患者有较低的初始白细胞计数和外周细胞。AML-M7亚型在DS患者中比在非DS患者中更常见(分别为80.5%比9.1%,p = 0.05和13.7%比18.4%,p = 0.03)。与非退行性痴呆患者相比,退行性痴呆患者的早期死亡率和治疗相关死亡率明显更高(30.3%比13.5%,p p = 0.02)。在研究期间,退行性痴呆患者的早期死亡率有所下降,生存率有所提高,这表明化疗方案和支持性护理随着时间的推移有所改善。
Survival outcomes of myeloid leukemia associated with Down syndrome and de novo acute myeloid leukemia in children: Experience from a single tertiary center in Thailand.
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%, p < 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%, p = 0.05 and 13.7% vs. 18.4%, p = 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%, p < 0.01 and 39.4% vs. 19.5%, p = 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.
期刊介绍:
PHO: Pediatric Hematology and Oncology covers all aspects of research and patient management within the area of blood disorders and malignant diseases of childhood. Our goal is to make PHO: Pediatric Hematology and Oncology the premier journal for the international community of clinicians and scientists who together aim to define optimal therapeutic strategies for children and young adults with cancer and blood disorders. The journal supports articles that address research in diverse clinical settings, exceptional case studies/series that add novel insights into pathogenesis and/or clinical care, and reviews highlighting discoveries and challenges emerging from consortia and conferences. Clinical studies as well as basic and translational research reports regarding cancer pathogenesis, genetics, molecular diagnostics, pharmacology, stem cells, molecular targeting, cellular and immune therapies and transplantation are of interest. Papers with a focus on supportive care, late effects and on related ethical, legal, psychological, social, cultural, or historical aspects of these fields are also appreciated. Reviews on important developments in the field are welcome. Articles from scientists and clinicians across the international community of Pediatric Hematology and Oncology are considered for publication. The journal is not dependent on or connected with any organization or society. All submissions undergo rigorous peer review prior to publication. Our Editorial Board includes experts in Pediatric Hematology and Oncology representing a wide range of academic and geographic diversity.