Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2018 Aug 16;44(1):94.
doi: 10.1186/s13052-018-0541-6.

ETV6/RUNX1-positive childhood acute lymphoblastic leukemia in China: excellent prognosis with improved BFM protocol

Yu Wang  1 , Hui-Min Zeng  2 , Le-Ping Zhang  1
Affiliations

ETV6/RUNX1-positive childhood acute lymphoblastic leukemia in China: excellent prognosis with improved BFM protocol

Yu Wang et al. Ital J Pediatr. .

Abstract

Background: In childhood B-precursor acute lymphoblastic leukemia (B-ALL), the ETV6/RUNX1 fusion transcript is considered to have an excellent outcome. However, few studies of children with ETV6/RUNX1-positive ALL from China have been conducted. It is largely unknown whether clinical outcomes for patients with this genotype and important factors that influence such outcomes are similar to those reported in other countries. Therefore, it is important to analyze the outcomes of children with ETV6/RUNX1-positive ALL treated at our institution with the aim of identifying significant prognostic variables in a Chinese population.

Methods: We studied the clinical characteristics and treatment outcomes for 77 pediatric patients diagnosed with ETV6/RUNX1-positive ALL between 2005 and 2015 at our institution.

Results: The 5-year event-free survival (EFS) and the disease-free survival (DFS) were reported to be 90% ± 3% and 96% ± 3% respectively. Two patients had a relapse at a median of 42 months from diagnosis and the 5-year cumulative incidence of relapse was 2.1%. Despite intensive chemotherapy or allogeneic hematopoietic cell transplantation, the 2 relapsed patients succumbed to the disease progression and the 5-year overall survival (OS) was 97% ± 2%. Multivariate analysis for EFS revealed that the minimal residual disease (MRD) ≥10- 3 on Day + 33 negatively affected the outcome.

Conclusions: In conclusion, patients with ETV6/RUNX1 fusion transcript can achieve a high rate of complete remission and the long-term curative effect was excellent under risk-stratified treatment. In case of relapse, the MRD level at the end of induction therapy should be taken into consideration while deciding the appropriate chemotherapy dosage.

Keywords: Acute lymphoblastic leukemia; ETV6/RUNX1; MRD; Prognosis.

PubMed Disclaimer

Conflict of interest statement

Ethics approval and consent to participate

This study was approved by the ethics committee of our intistuion.

This manuscript is not under simultaneous consideration by any other publication.

Informed consent was obtained from the parent or guardian and assent obtained from the patient when appropriate.

Consent for publication

Informed consent was obtained from the parent or guardian and assent obtained from the patient when appropriate.

Competing interests

The authors declare that they have no competing interest.

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Figures

Fig. 1
Fig. 1
Flow chart of the improved BFM protocol at our institution. It includes remission induction, consolidation treatment, and maintenance therapy according to risk group
Fig. 2
Fig. 2
Kaplan-Meier estimates of OS (a), DFS (b) and EFS (c) in ETV6/RUNX1 pediatric ALL patients by study. Rates at 5 years are reported as means ± standard errors. The probability of 5-year OS, DFS and EFS were reported to be (97 ± 2) %, (96 ± 3) %, and (90 ± 3) %, respectively
See this image and copyright information in PMC

References

    1. Pui CH, Evans WE. Treatment of acute lymphoblastic leukemia [J] N Engl J Med. 2006;354(2):166–178. doi: 10.1056/NEJMra052603. - DOI - PubMed
    1. Stanulla M, Schrappe M. Treatment of childhood acute lymphoblastic leukemia [J] Semin Hematol. 2009;46(1):52–63. doi: 10.1053/j.seminhematol.2008.09.007. - DOI - PubMed
    1. Shurtleff SA, Buijs A, Behm FG, Rubnitz JE, Raimondi SC, Hancock ML, et al. TEL/AML1 fusion resulting from a cryptic t (12; 21) is the most common genetic lesion in pediatric ALL and defines a subgroup of patients with an excellent prognosis [J] Leukemia. 1995;9(12):1985–1989. - PubMed
    1. Romana SP, Mauchauffé M, Le Coniat M, Chumakov I, Le Paslier D, Berger R, et al. The t (12; 21) of acute lymphoblastic leukemia results in a tel-AML1 gene fusion [J] Blood. 1995;85(12):3662–3670. - PubMed
    1. Borkhardt A, Cazzaniga G, Viehmann S, Valsecchi MG, Ludwig WD, Burci L, et al. Incidence and clinical relevance of TEL/AML1 fusion genes in children with acute lymphoblastic leukemia enrolled in the German and Italian multicenter therapy trials. Associazione Italiana Ematologia Oncologia Pediatrica and the berlin-Frankfurt-Munster study group [J] Blood. 1997;90(2):571–577. - PubMed

MeSH terms

Supplementary concepts

LinkOut - more resources