title: Prospective analysis of TEL gene rearrangements in childhood acute lymphoblastic leukemia: a Children's Oncology Group study.
abstract: OBJECTIVE
  To prospectively determine the prognostic significance of the TEL-AML1 fusion in children with acute lymphoblastic leukemia (ALL).
  
  
  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).
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Oncology, Mail Stop 260, St Jude Children's Research Hospital, 332 N Lauderdale St, Memphis, TN 38105-2794, USA. jeffrey.rubnitz@stjude.org
authors.forename: Jeffrey E
authors.initials: JE
authors.lastname: Rubnitz
authors.forename: David
authors.initials: D
authors.lastname: Wichlan
authors.forename: Meenakshi
authors.initials: M
authors.lastname: Devidas
authors.forename: Jonathan
authors.initials: J
authors.lastname: Shuster
authors.forename: Stephen B
authors.initials: SB
authors.lastname: Linda
authors.forename: Joanne
authors.initials: J
authors.lastname: Kurtzberg
authors.forename: Beverly
authors.initials: B
authors.lastname: Bell
authors.forename: Stephen P
authors.initials: SP
authors.lastname: Hunger
authors.forename: Allen
authors.initials: A
authors.lastname: Chauvenet
authors.forename: Ching-Hon
authors.initials: CH
authors.lastname: Pui
authors.forename: Bruce
authors.initials: B
authors.lastname: Camitta
authors.forename: Jeanette
authors.initials: J
authors.lastname: Pullen
mesh_terms: D000293:Adolescent; D000328:Adult; D001853:Bone Marrow / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D050676:Core Binding Factor Alpha 2 Subunit / Q000235:genetics*; D018572:Disease-Free Survival; D005260:Female; D015973:Gene Expression Regulation, Leukemic; D015321:Gene Rearrangement; D006801:Humans; D007223:Infant; D053208:Kaplan-Meier Estimate; D008297:Male; D015514:Oncogene Proteins, Fusion / Q000235:genetics*; D015452:Precursor B-Cell Lymphoblastic Leukemia-Lymphoma / Q000235:genetics* / Q000401:mortality / Q000473:pathology / Q000628:therapy; D011379:Prognosis; D016016:Proportional Hazards Models; D011446:Prospective Studies; D018570:Risk Assessment; D012737:Sex Factors; D015996:Survival Rate; D013997:Time Factors; D016896:Treatment Outcome; D014481:United States; D000097764:ETS Translocation Variant 6 Protein
chemical_list: D050676:Core Binding Factor Alpha 2 Subunit; D015514:Oncogene Proteins, Fusion; C102421:TEL-AML1 fusion protein; D000097764:ETS Translocation Variant 6 Protein