Prospective analysis of TEL gene rearrangements in childhood acute lymphoblastic leukemia: a Children's Oncology Group study.

Journal Article (Journal Article;Multicenter Study)

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.

Full Text

Duke Authors

Cited Authors

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

Published Date

  • May 1, 2008

Published In

Volume / Issue

  • 26 / 13

Start / End Page

  • 2186 - 2191

PubMed ID

  • 18445843

Pubmed Central ID

  • PMC4485397

Electronic International Standard Serial Number (EISSN)

  • 1527-7755

Digital Object Identifier (DOI)

  • 10.1200/JCO.2007.14.3552


  • eng

Conference Location

  • United States