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1 "Dae-Chul Jeong"
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Original Article
Outcome and Prognostic Factors for ETV6/RUNX1 Positive Pediatric Acute Lymphoblastic Leukemia Treated at a Single Institution in Korea
Jae Wook Lee, Seong-koo Kim, Pil-Sang Jang, Nack-Gyun Chung, Dae-Chul Jeong, Myungshin Kim, Bin Cho, Hack-Ki Kim
Cancer Res Treat. 2017;49(2):446-453.   Published online August 10, 2016
DOI: https://doi.org/10.4143/crt.2016.211
AbstractAbstract PDFSupplementary MaterialPubReaderePub
Purpose
ETV6/RUNX1 (+) acute lymphoblastic leukemia (ALL), which is the most common genetic subtype of pediatric ALL, has a favorable prognosis. In this study, we analyzed the outcome of ETV6/RUNX1 (+) ALL patients treated at our institution with the aim of identifying significant prognostic variables.
Materials and Methods
Sixty-three patients were diagnosed with ETV6/RUNX1 (+) ALL from 2005 to 2011. Prognostic variables studied included minimal residual disease (MRD) as detected by ETV6/RUNX1 (+) fusion, and the presence of additional cytogenetic abnormalities.
Results
The 5-year event-free survival was 84.1±4.6%, with 10 patients relapsing at a median of 28.3 months from diagnosis for a 5-year cumulative incidence of relapse of 15.9±4.6%. Multivariate analysis revealed that the presence MRD, as detected by real-time quantitative-polymerase chain reaction or fluorescence in situ hybridization for ETV6/RUNX1 fusion at end of remission induction, and the presence of additional structural abnormalities of 12p (translocations or inversions) negatively affected outcome. Despite treatment such as allogeneic hematopoietic cell transplantation, eight of the 10 relapsed patients died from disease progression for overall survival of 82.5±6.9%.
Conclusion
ETV6/RUNX1 ALL may be heterogeneous in terms of prognosis, and variables such as MRD at end ofremission induction or additional structural abnormalities of 12p could define a subset of patients who are likely to have poor outcome.

Citations

Citations to this article as recorded by  
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  • Outcome and Prognostic Factors of Haploidentical Allogeneic Hematopoietic Stem Cell Transplantation in Pediatric Relapsed or Refractory ETV6/RUNX1-Positive Acute Lymphoblastic Leukemia
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    Acta Haematologica.2024; 147(5): 534.     CrossRef
  • Differing Outcomes of Patients with High Hyperdiploidy and ETV6-RUNX1 Rearrangement in Korean Pediatric Precursor B Cell Acute Lymphoblastic Leukemia
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  • Prognostic Value and Outcome for ETV6/RUNX1-Positive Pediatric Acute Lymphoblastic Leukemia: A Report From the South China Children’s Leukemia Group
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    Frontiers in Oncology.2021;[Epub]     CrossRef
  • ETV6/RUNX1-positive childhood acute lymphoblastic leukemia in China: excellent prognosis with improved BFM protocol
    Yu Wang, Hui-min Zeng, Le-ping Zhang
    Italian Journal of Pediatrics.2018;[Epub]     CrossRef
  • Prognostic factors and treatment of pediatric acute lymphoblastic leukemia
    Jae Wook Lee, Bin Cho
    Korean Journal of Pediatrics.2017; 60(5): 129.     CrossRef
  • ETV6 and ETV7: Siblings in hematopoiesis and its disruption in disease
    Parisa Rasighaemi, Alister C. Ward
    Critical Reviews in Oncology/Hematology.2017; 116: 106.     CrossRef
  • Pathogenesis of ETV6/RUNX1-positive childhood acute lymphoblastic leukemia and mechanisms underlying its relapse
    Congcong Sun, Lixian Chang, Xiaofan Zhu
    Oncotarget.2017; 8(21): 35445.     CrossRef
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