Please use this identifier to cite or link to this item: http://hdl.handle.net/2440/117025
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Type: Journal article
Title: Acute lymphoblastic leukemia in children with Down syndrome: a retrospective analysis from the Ponte di Legno study group
Author: Buitenkamp, T.D.
Izraeli, S.
Zimmermann, M.
Forestier, E.
Heerema, N.A.
van den Heuvel-Eibrink, M.M.
Pieters, R.
Korbijn, C.M.
Silverman, L.B.
Schmiegelow, K.
Liang, D.-.C.
Horibe, K.
Arico, M.
Biondi, A.
Basso, G.
Rabin, K.R.
Schrappe, M.
Cario, G.
Mann, G.
Morak, M.
et al.
Citation: BLOOD, 2014; 123(1):70-77
Publisher: American Society of Hematology
Issue Date: 2014
ISSN: 0006-4971
1528-0020
Statement of
Responsibility: 
Trudy D. Buitenkamp, Shai Izraeli, Martin Zimmermann, Erik Forestier, Nyla A. Heerema ... Charles G. Mullighan ... et al.
Abstract: Children with Down syndrome (DS) have an increased risk of B-cell precursor (BCP) acute lymphoblastic leukemia (ALL). The prognostic factors and outcome of DS-ALL patients treated in contemporary protocols are uncertain. We studied 653 DS-ALL patients enrolled in 16 international trials from 1995 to 2004. Non-DS BCP-ALL patients from the Dutch Child Oncology Group and Berlin-Frankfurt-Münster were reference cohorts. DS-ALL patients had a higher 8-year cumulative incidence of relapse (26% ± 2% vs 15% ± 1%, P < .001) and 2-year treatment-related mortality (TRM) (7% ± 1% vs 2.0% ± <1%, P < .0001) than non-DS patients, resulting in lower 8-year event-free survival (EFS) (64% ± 2% vs 81% ± 2%, P < .0001) and overall survival (74% ± 2% vs 89% ± 1%, P < .0001). Independent favorable prognostic factors include age <6 years (hazard ratio [HR] = 0.58, P = .002), white blood cell (WBC) count <10 × 10(9)/L (HR = 0.60, P = .005), and ETV6-RUNX1 (HR = 0.14, P = .006) for EFS and age (HR = 0.48, P < .001), ETV6-RUNX1 (HR = 0.1, P = .016) and high hyperdiploidy (HeH) (HR = 0.29, P = .04) for relapse-free survival. TRM was the major cause of death in ETV6-RUNX1 and HeH DS-ALLs. Thus, while relapse is the main contributor to poorer survival in DS-ALL, infection-associated TRM was increased in all protocol elements, unrelated to treatment phase or regimen. Future strategies to improve outcome in DS-ALL should include improved supportive care throughout therapy and reduction of therapy in newly identified good-prognosis subgroups.
Keywords: Karyotyping
Rights: © 2014 by The American Society of Hematology
RMID: 0030061359
DOI: 10.1182/blood-2013-06-509463
Appears in Collections:Paediatrics publications

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