title: Outcome of treatment in childhood acute lymphoblastic leukaemia with rearrangements of the 11q23 chromosomal region.
abstract: BACKGROUND
  The prognosis and optimum treatment of childhood acute lymphoblastic leukaemia (ALL) with abnormalities of chromosomal band 11q23 are controversial. We aimed to identify prognostic factors that might help in planning future therapy, and to assess the effectiveness of haemopoietic stem-cell transplantation in patients with the t(4;11) translocation, which is associated with a particularly poor outcome.
  
  
  METHODS
  We reviewed data on 497 children and young adults who had ALL with various 11q23 abnormalities, including the translocations t(4;11), t(9;11), and t(11;19). All patients were treated with intensive chemotherapy, with or without haemopoietic stem-cell transplantation in first complete remission, by 11 study groups and single institutions from 1983 to 1995.
  
  
  RESULTS
  Age was the most important prognostic factor. In a Cox's proportional-hazard model stratified by 11q23 abnormalities, infants younger than 1 year fared significantly worse than patients 1 year of age or older (hazard ratio for event-free survival 1 84 [95% CI 1 38-2 47], p=0 0001). Among infants, any category of 11q23 abnormality conferred a dismal outcome, whereas in older patients, t(4;11) and t(9;11) were associated with a worse outcome than were other 11q23 changes. In the largest subgroup--256 patients with t(4;11)--any type of transplantation was associated with significantly worse disease-free survival (1 61 [1 10-2 35], p=0 014) and overall survival (1 76 [1 08-2 45], p=0 004) compared with chemotherapy only. Even transplantation with stem cells from HLA-matched related or HLA-matched unrelated donors tended to be associated with a worse outcome than chemotherapy alone.
  
  
  CONCLUSIONS
  The prognosis of acute lymphoblastic leukaemia with an 11q23 abnormality is particularly dismal in infants. Allogeneic transplantation with haemopoietic stem cells from an HLA-matched related donor does not seem to improve the clinical outcome in patients with t(4;11)-positive leukaemia.
authors.affiliation: St Jude Children's Research Hospital and the University of Tennessee, College of Medicine, Memphis, TN 38105, USA. ching-hon.pui@stjude.org
authors.forename: Ching-Hon
authors.initials: CH
authors.lastname: Pui
authors.forename: Paul S
authors.initials: PS
authors.lastname: Gaynon
authors.forename: James M
authors.initials: JM
authors.lastname: Boyett
authors.forename: Judith M
authors.initials: JM
authors.lastname: Chessells
authors.forename: André
authors.initials: A
authors.lastname: Baruchel
authors.forename: Willem
authors.initials: W
authors.lastname: Kamps
authors.forename: Lewis B
authors.initials: LB
authors.lastname: Silverman
authors.forename: Andrea
authors.initials: A
authors.lastname: Biondi
authors.forename: Dörthe O
authors.initials: DO
authors.lastname: Harms
authors.forename: Etienne
authors.initials: E
authors.lastname: Vilmer
authors.forename: Martin
authors.initials: M
authors.lastname: Schrappe
authors.forename: Bruce
authors.initials: B
authors.lastname: Camitta
mesh_terms: D017677:Age Distribution; D000970:Antineoplastic Agents / Q000627:therapeutic use; D016009:Chi-Square Distribution; D002648:Child; D002675:Child, Preschool; D002880:Chromosomes, Human, Pair 11 / Q000235:genetics*; D018572:Disease-Free Survival; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007223:Infant; D008297:Male; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000235:genetics* / Q000628:therapy; D011379:Prognosis; D012189:Retrospective Studies; D014178:Translocation, Genetic; D016896:Treatment Outcome
chemical_list: D000970:Antineoplastic Agents