[The Prognostic Factors for AML Children with CBFβ/MYH11 Positive]

Zhongguo Shi Yan Xue Ye Xue Za Zhi. 2021 Apr;29(2):369-373. doi: 10.19746/j.cnki.issn.1009-2137.2021.02.010.
[Article in Chinese]

Abstract

Objective: To analyze the prognostic factors of AML children with CBFβ/MYH11 positive.

Methods: Twenty-eight children with CBFβ/MYH11 positive treated in our hospital from May 2012 to June 2018 were selected, the clinical data and curative were analyzed and evaluated.

Results: Five-year OS and 5-year EFS rate of CBFβ/MYH11 positive AML children was 76.8% and 64.0% efficacy, respectively. Univariate analysis results showed that the OS rate of CBFβ/MYH11 positive AML children with WBC<60.0×109/L was 86.5%, which was significantly higher than those of CBFβ/MYH11 positive AML children with WBC≥60.0×109/L (χ2=3.891, P<0.05). The EFS rate of CBFβ/MYH11 positive AML children with WBC<60.0×109/L was 76.0%, which was significantly higher than those of AML children with WBC≥60.0×109/L (χ2=4.588, P<0.05). The EFS rate of CBFβ/MYH11 positive AML children with XRCC-Thr241Met wild type was 77.9%, which was significantly higher than those of AML children with XRCC-Thr241Met variant (χ2=3.960, P<0.05). Cox multivariate survival analysis results showed that WBC level at initial diagnosis was the risk factor for OS rate. The WBC level and XRCC-Thr241Met type at initial diagnosis were the risk factors for EFS rate.

Conclusion: WBC level and XRCC-Thr241Met genotype at initial diagnosis are the major affecting factors for prognosis of AML children with CBFβ/MYH11 positive.

题目: 伴CBFβ/MYH11阳性初诊AML患儿的预后影响因素分析.

目的: 分析伴CBFβ/MYH11阳性初诊急性髓系白血病(AML)患儿的预后影响因素.

方法: 选取2012年5月至2018年6月本院收治的原发性初治伴inv(16)/ CBFβ-MYH11阳性AML患儿28例,对其临床资料及治疗效果等进行分析和评估.

结果: 所有患儿5年总生存(OS)率76.8%,5年无事件生存(EFS)率64.0%。单因素分析结果显示,WBC<60.0×109/L的患儿OS率为86.5%,显著性高于WBC≥60.0×109/L的患儿的50.0%(χ2=3.891,P<0.05)。WBC <60.0×109/L的患儿EFS率为76.0%,显著性高于WBC≥60.0×109/L的患儿的37.5%(χ2=4.588,P<0.05)。XRCC-Thr241Met野生型患儿EFS率为77.9%,显著性高于XRCC-Thr241Met变异型患儿的43.6%(χ2= 3.960,P<0.05)。Cox多因素生存分析显示,初诊WBC水平为影响该类患儿OS率的危险因素,初诊WBC水平及XRCC-Thr241Met是否野生型为影响该类患儿EFS率的危险因素.

结论: 初诊WBC水平及XRCC-Thr241Met基因型为影响伴CBFβ/MYH11阳性初诊AML患儿预后的主要因素.

MeSH terms

  • Child
  • Chromosome Inversion*
  • Genotype
  • Humans
  • Leukemia, Myeloid, Acute* / genetics
  • Myosin Heavy Chains
  • Oncogene Proteins, Fusion
  • Prognosis

Substances

  • CBFbeta-MYH11 fusion protein
  • MYH11 protein, human
  • Oncogene Proteins, Fusion
  • Myosin Heavy Chains