[核心结合因子急性髓性白血病的临床特征和预后:中国南方儿童多中心研究]
1Department of Pediatrics, the First Affiliated Hospital of Xiamen University, Xiamen 361003, China.
Zhonghua er ke za zhi = Chinese journal of pediatrics
|October 7, 2023
概括
儿童中核结合因子 (CBF) 急性髓性白血病 (AML) 显示出比非CBF-AML更好的结果. 在CBF-AML中常见的AML1-ETO亚型,具有更高的外骨膜干涉和染色体异常,但预后与CBFβ-MYH11相似.
科学领域:
- 儿科血液学 瘤学 儿科血液学
- 分子遗传学 分子遗传学
- 临床瘤学临床瘤学
背景情况:
- 核心结合因子 (CBF) 急性髓性白血病 (AML) 是儿童的一个明显的亚型.
- 了解CBF-AML的临床特征,治疗疗效和预后因素对于改善结果至关重要.
研究的目的:
- 分析中国南部儿科CBF-AML的临床特征,治疗疗效和预后因素.
- 将CBF-AML与非CBF-AML进行比较,并研究CBF-AML亚组 (AML1-ETO和CBFβ-MYH11) 之间的差异.
主要方法:
- 对584名儿科AML患者进行了回顾性队列研究.
- 根据融合基因结果,患者被分为CBF-AML (n=189) 和非CBF-AML (n=395) 组.
- 使用卡普兰-梅尔和考克斯回归模型进行了生存分析.
主要成果:
- 与非CBF-AML儿童相比,CBF-AML儿童的完全缓解率 (91.0%vs. 78.0%),复发率 (10.1%vs. 18.7%) 和死亡率 (13.2%vs. 25.6%) 显著更高,而非CBF-AML儿童的复发率更低.
- 美国AML1-ETO亚组比CBFβ-MYH11亚组显示出更多的外骨膜入侵和额外的染色体异常.
- 五年无事件生存率 (EFS) 和整体生存率 (OS) 在CBF-AML组 (77.0%和83.7%) 与非CBF-AML组 (61.9%和67.3%) 相比显著更好.
结论:
- 在儿童中,CBF-AML的预后比非CBF-AML更好.
- AML1-ETO亚型比AML1-ETO亚型更为普遍,并且与更高的外骨髓干干涉和染色体异常有关,但其预后与CBFβ-MYH11相似.
- FLAG-IDA诱导疗法可以改善儿科CBF-AML的预后,具有高白细胞计数和额外的染色体异常.
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