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Outcome of treatment in childhood acute lymphoblastic leukaemia with rearrangements of the 11q23 chromosomal region. Lancet 2002 Jun 01;359(9321):1909-15

Date

06/12/2002

Pubmed ID

12057554

DOI

10.1016/S0140-6736(02)08782-2

Scopus ID

2-s2.0-0036606506 (requires institutional sign-in at Scopus site)   316 Citations

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.

FINDINGS: 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.

INTERPRETATION: 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.

Author List

Pui CH, Gaynon PS, Boyett JM, Chessells JM, Baruchel A, Kamps W, Silverman LB, Biondi A, Harms DO, Vilmer E, Schrappe M, Camitta B



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

Age Distribution
Antineoplastic Agents
Chi-Square Distribution
Child
Child, Preschool
Chromosomes, Human, Pair 11
Disease-Free Survival
Female
Hematopoietic Stem Cell Transplantation
Humans
Infant
Male
Precursor Cell Lymphoblastic Leukemia-Lymphoma
Prognosis
Retrospective Studies
Translocation, Genetic
Treatment Outcome