Cancer Res Treat.  2021 Apr;53(2):567-575. 10.4143/crt.2020.507.

Differing Outcomes of Patients with High Hyperdiploidy and ETV6-RUNX1 Rearrangement in Korean Pediatric Precursor B Cell Acute Lymphoblastic Leukemia

Affiliations
  • 1Division of Hematology and Oncology, Department of Pediatrics, College of Medicine, The Catholic University of Korea, Seoul, Korea

Abstract

Purpose
Recent cooperative trials in pediatric acute lymphoblastic leukemia (ALL) report long-term event-free survival (EFS) of greater than 80%. In this study, we analyzed the outcome and prognostic factors for patients with precursor B cell ALL (n=405) diagnosed during a 10-year period (2005-2015) at our institution.
Materials and Methods
All patients were treated with a uniform institutional regimen based on four risk groups, except for steroid type; patients diagnosed up till 2008 receiving dexamethasone, while subsequent patients received prednisolone. None of the patients received cranial irradiation in first complete remission.
Results
The 10-year EFS and overall survival was 76.3%±2.3% and 85.1%±1.9%. Ten-year cumulative incidence of relapse, any central nervous system (CNS) relapse and isolated CNS relapse was 20.8%±2.2%, 3.7%±1.1% and 2.5%±0.9% respectively. A comparison of established, good prognosis genetic abnormalities showed that patients with high hyperdiploidy had significantly better EFS than those with ETV6-RUNX1 rearrangement (10-year EFS of 91.2%±3.0% vs. 79.5%±4.4%, p=0.033). For the overall cohort, male sex, infant ALL, initial CNS involvement, and Philadelphia chromosome (+) ALL were significant factors for lower EFS in multivariate study, while high hyperdiploidy conferred favorable outcome. For high and very high risk patients (n=231), high hyperdiploidy was the only significant factor for EFS in multivariate study.
Conclusion
Regarding good prognosis genetic abnormalities, patients with high hyperdiploidy had significantly better outcome than ETV6-RUNX1 (+) patients. High hyperdiploidy was a major, favorable prognostic factor in the overall patient group, as well as the subgroup of patients with higher risk.

Keyword

Precursor cell lymphoblastic leukemia-lymphoma; High hyperdiploidy; Child

Figure

  • Fig. 1 (A) The 10-year event-free survival (EFS) of overall cohort. (B) The 10-year overall survival (OS) of overall cohort.

  • Fig. 2 (A) Comparison of 10-year event-free survival (EFS) of patients with high hyperdiploidy (91.2%±3.0%) and ETV6-RUNX1 rearrangement (79.5%±4.4%). (B) The 10-year EFS of high hyperdiploidy patients according to risk group: low and standard risk 92.9%±3.4%; high and very high risk 88.2%±5.5%. (C) The 10-year EFS of ETV6-RUNX1(+) patients according to risk group: low and standard risk 86.1%±4.3%; high and very high risk 61.5%±10.8%.

  • Fig. 3 The 10-year overall survival of patients according to overall risk group: low risk, 95.7%±2.1%; standard risk, 98.8%±1.2%; high risk, 84.4%±3.7%, very high risk, 68.7%±4.6%.


Reference

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