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Search: WFRF:(Kowalczyk P.) > (2010-2014) > Acute lymphoblastic...

  • Buitenkamp, Trudy D. (author)

Acute lymphoblastic leukemia in children with Down syndrome : a retrospective analysis from the Ponte di Legno study group

  • Article/chapterEnglish2014

Publisher, publication year, extent ...

  • American Society of Hematology,2014
  • printrdacarrier

Numbers

  • LIBRIS-ID:oai:DiVA.org:umu-86068
  • https://urn.kb.se/resolve?urn=urn:nbn:se:umu:diva-86068URI
  • https://doi.org/10.1182/blood-2013-06-509463DOI

Supplementary language notes

  • Language:English
  • Summary in:English

Part of subdatabase

Classification

  • Subject category:ref swepub-contenttype
  • Subject category:art swepub-publicationtype

Notes

  • 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-Munster 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 x 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.

Subject headings and genre

Added entries (persons, corporate bodies, meetings, titles ...)

  • Izraeli, Shai (author)
  • Zimmermann, Martin (author)
  • Forestier, ErikUmeå universitet,Medicinsk och klinisk genetik(Swepub:umu)erfo0007 (author)
  • Heerema, Nyla A. (author)
  • van den Heuvel-Eibrink, Marry M. (author)
  • Pieters, Rob (author)
  • Korbijn, Carin M. (author)
  • Silverman, Lewis B. (author)
  • Schmiegelow, Kjeld (author)
  • Liang, Der-Cheng (author)
  • Horibe, Keizo (author)
  • Arico, Maurizio (author)
  • Biondi, Andrea (author)
  • Basso, Giuseppe (author)
  • Rabin, Karin R. (author)
  • Schrappe, Martin (author)
  • Cario, Gunnar (author)
  • Mann, Georg (author)
  • Morak, Maria (author)
  • Panzer-Grumayer, Renate (author)
  • Mondelaers, Veerle (author)
  • Lammens, Tim (author)
  • Cave, Helene (author)
  • Stark, Batia (author)
  • Ganmore, Ithamar (author)
  • Moorman, Anthony V. (author)
  • Vora, Ajay (author)
  • Hunger, Stephen P. (author)
  • Pui, Ching-Hon (author)
  • Mullighan, Charles G. (author)
  • Manabe, Atsushi (author)
  • Escherich, Gabriele (author)
  • Kowalczyk, Jerzy R. (author)
  • Whitlock, James A. (author)
  • Zwaan, C. Michel (author)
  • Umeå universitetMedicinsk och klinisk genetik (creator_code:org_t)

Related titles

  • In:Blood: American Society of Hematology123:1, s. 70-770006-49711528-0020

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