Outcome and Prognostic Factors for ETV6/RUNX1 Positive Pediatric Acute Lymphoblastic Leukemia Treated at a Single Institution in Korea.
- Author:
Jae Wook LEE
1
;
Seong koo KIM
;
Pil Sang JANG
;
Nack Gyun CHUNG
;
Dae Chul JEONG
;
Myungshin KIM
;
Bin CHO
;
Hack Ki KIM
Author Information
- Publication Type:Original Article
- Keywords: Acute lymphoblastic leukemia; ETV6/RUNX1; Minimal residual disease; 12p abnormalities
- MeSH: 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
- From:Cancer Research and Treatment 2017;49(2):446-453
- CountryRepublic of Korea
- Language:English
- 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.
