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

• Lee JW
• Kim S
• Jang PS
• Chung NG
• Cho B

• KMID: 2514937
• DOI: http://doi.org/10.4143/crt.2020.507

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

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

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  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%.

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  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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