Faculty Collaboration Database

Prospective analysis of TEL gene rearrangements in childhood acute lymphoblastic leukemia: a Children's Oncology Group study. J Clin Oncol 2008 May 01;26(13):2186-91

Date

05/01/2008

Pubmed ID

18445843

Pubmed Central ID

PMC4485397

DOI

10.1200/JCO.2007.14.3552

Scopus ID

2-s2.0-43749088252 (requires institutional sign-in at Scopus site)   73 Citations

Abstract

PURPOSE: To prospectively determine the prognostic significance of the TEL-AML1 fusion in children with acute lymphoblastic leukemia (ALL).

PATIENTS AND METHODS: TEL gene status was determined for 926 patients with B-precursor ALL enrolled on the Pediatric Oncology Group ALinC 16 trials and patients were observed for a median time of 8 years.

RESULTS: Rearrangements of the TEL gene were detected in 244 patients (26%). The estimated 5-year event-free survival rate (+/- SE) for patients with TEL rearrangements was 86% +/- 2%, compared with 72% +/- 2% for those with germline TEL (P < .0001). TEL rearrangements were associated with a superior outcome among patients with standard-risk ALL, high-risk ALL, and rapid early responses to therapy. In a multivariate analysis that included risk group, sex, and day 15 marrow status, TEL status was an independent predictor of outcome (P = .0002).

CONCLUSION: We conclude that TEL gene status should be incorporated into risk classification schemes and suggest that patients who have standard-risk features, the TEL-AML1 fusion, and rapid early responses to therapy, should be treated with antimetabolite-based therapy designed to maintain their high cure rates and avoid late effects.

Author List

Rubnitz JE, Wichlan D, Devidas M, Shuster J, Linda SB, Kurtzberg J, Bell B, Hunger SP, Chauvenet A, Pui CH, Camitta B, Pullen J, Children's Oncology Group

Author

Bruce m. Camitta Professor in the Pediatrics department at Medical College of Wisconsin

MESH terms used to index this publication - Major topics in bold

Adolescent
Adult
Bone Marrow
Child
Child, Preschool
Core Binding Factor Alpha 2 Subunit
Disease-Free Survival
Female
Gene Expression Regulation, Leukemic
Gene Rearrangement
Humans
Infant
Kaplan-Meier Estimate
Male
Oncogene Proteins, Fusion
Precursor B-Cell Lymphoblastic Leukemia-Lymphoma
Prognosis
Proportional Hazards Models
Prospective Studies
Risk Assessment
Sex Factors
Survival Rate
Time Factors
Treatment Outcome
United States