Cancer Res Treat.  2017 Apr;49(2):446-453. 10.4143/crt.2016.211.

Outcome and Prognostic Factors for ETV6/RUNX1 Positive Pediatric Acute Lymphoblastic Leukemia Treated at a Single Institution in Korea

• Lee JW
• Kim SK
• Jang PS
• Chung NG
• Jeong DC
• Kim M
• Cho B
• Kim HK

• KMID: 2378117
• DOI: http://doi.org/10.4143/crt.2016.211

Abstract

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.

Keyword

Acute lymphoblastic leukemia; ETV6/RUNX1; Minimal residual disease; 12p abnormalities

MeSH Terms

Cell Transplantation
Chromosome Aberrations
Diagnosis
Disease Progression
Disease-Free Survival
Fluorescence
Humans
In Situ Hybridization
Incidence
Korea*
Multivariate Analysis
Neoplasm, Residual
Precursor Cell Lymphoblastic Leukemia-Lymphoma*
Prognosis
Recurrence
Remission Induction
Transplants

Figure

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  Fig. 1. (A) Event-free survival (EFS) of the ETV6/RUNX1 (+) acute lymphoblastic leukemia (ALL) cohort. (B) EFS of the ETV6/RUNX1 (+) ALL cohort according to overall risk group.

Reference

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