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[Clinical features and prognosis of core binding factor acute myeloid leukemia in children]

Chao Liu1, Xiao-Yan Chen, Mei-Hui Yi

  • 1State Key Laboratory of Experimental Hematology, National Clinical Research Center for Blood Diseases, Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Tianjin 300020, China. xfzhu@ihcams.ac.cn.

Zhongguo Dang Dai Er Ke Za Zhi = Chinese Journal of Contemporary Pediatrics
|July 17, 2020
PubMed

Insights

Core binding factor acute myeloid leukemia (CBF-AML) in children is often associated with a good prognosis. The AML1-ETO fusion gene is most common, and its prognosis is similar to CBFB-MYH11.

Area of Science:

  • Pediatric Hematology
  • Oncology
  • Genetics

Background:

  • Core binding factor acute myeloid leukemia (CBF-AML) is a subtype of acute myeloid leukemia.
  • CBF-AML is characterized by specific chromosomal translocations involving the core binding factor (CBF) gene complex.

Purpose of the Study:

  • To investigate the clinical features and prognosis of pediatric CBF-AML.
  • To compare outcomes between different fusion gene types (AML1-ETO and CBFB-MYH11) in children with CBF-AML.

Main Methods:

  • Retrospective analysis of chart data from 91 newly diagnosed pediatric CBF-AML patients.
  • Patients were categorized into AML1-ETO and CBFB-MYH11 groups based on fusion gene type.
  • Clinical features and survival rates (event-free survival and overall survival) were analyzed.

Main Results:

  • AML1-ETO (81%) was more common than CBFB-MYH11 (19%).
  • Deletion of sex chromosome was the most frequent additional chromosomal abnormality (31%).
  • High complete remission rate (97%) and favorable 5-year event-free survival (65%) and overall survival (75%) were observed. No significant prognostic difference between AML1-ETO and CBFB-MYH11 groups.

Conclusions:

  • AML1-ETO is the predominant fusion gene in pediatric CBF-AML.
  • Pediatric CBF-AML generally carries a favorable prognosis.
  • Patients with AML1-ETO fusion gene have a similar prognosis to those with CBFB-MYH11.
Abstract

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