Abstract

Relapsed/refractory acute lymphoblastic leukemia (ALL) is a leading cause of death by cancer in children. Our institution has switched relapse treatment strategy to improve survival. We reviewed records of first relapse/refractory childhood ALL between 1996 and 2012. Based on length of first remission, relapse site and immunophenotype, patients were classified into two groups: standard-risk relapse (SRR) and high-risk relapse and refractory (HRRR). Before 2007, all patients were uniformly treated with the same induction as at presentation, followed by hematopoietic stem cell transplantation (HSCT). Since 2007, treatment was given according to risk of failure: SRR were mostly treated with chemotherapy; HRRR patients underwent HSCT after intensive chemotherapy, aiming reduction of pre-transplant disease burden. Sixty-four patients were included. Thirty (47%) were SRR and 34 (53%) HRRR, including 11 with refractory ALL. Five-years overall survival (OS) and event-free survival (EFS) were similar for SRR, but were significantly higher with new risk-based strategy for HRRR: 56% versus 17% (P = 0.03) for OS, and 56% vs 11% for EFS (P = 0.008), respectively. In multivariate analysis, treatment strategy was significantly associated with survival. In conclusion, change for a risk-based strategy in our institution increased survival of high-risk patients to levels similar of those of standard-risk patients.

Highlights

  • Acute lymphoblastic leukemia (ALL) is the most common pediatric malignancy

  • We reviewed the records of all patients with refractory or first relapse ALL aged from 1 to 18 years at initial diagnosis, having begun their first-line chemotherapy at our institution (Sainte Justine University Health Center, SJUHC) between January 1996 and December 2011, and having relapsed until December 2012

  • Exclusions criteria were a diagnosis of mature B-cell leukemia, ALL associated with a genetic background (i.e. Down syndrome, ataxia-telangiectasia), secondary ALL

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Summary

Introduction

Acute lymphoblastic leukemia (ALL) is the most common pediatric malignancy. Long-term survival has become the reality for the majority of children diagnosed with ALL, with approximately 85% [1, 2] surviving 5 years or longer after diagnosis. Depending on certain risk factors, such as age at diagnosis, white blood cell (WBC) count at presentation, hematopoietic lineage, and cytogenetic abnormalities, approximately 15% will experience relapse, making recurrent ALL a relatively common situation in pediatric hemato-oncology [3]. PLOS ONE | DOI:10.1371/journal.pone.0160310 September 15, 2016

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