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Optimal treatment intensity in children with Down syndrome and myeloid leukaemia: data from 56 children treated on NOPHO-AML protocols and a review of the literature

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Abstract

Children with Down syndrome (DS) and myeloid leukaemia have a significantly higher survival rate than other children, but they also experience considerable treatment-related toxicity. We analysed data on 56 children with DS who were treated on the Nordic Society for Paediatric Haematology and Oncology-acute myeloid leukaemia (NOPHO-AML)88 and NOPHO-AML93 protocols and reviewed the literature. In the dose-intensive NOPHO-AML88 protocol, 8 out of 15 patients (53%) experienced an event. In the less dose-intensive NOPHO-AML93 protocol, 7 out of 41 patients (17%) had an event. Therapy was reduced in 29 patients (52%) with in average 75% and 67% of the scheduled dose of anthracycline and cytarabine, respectively. Treatment-related death occurred in seven who all received full treatment. Relapse and resistant disease occurred at a similar rate in those receiving full and reduced treatment. Review of major series of myeloid leukaemia of DS showed no clear relationship between dose and survival; however, it appears that both a reduction in treatment dose and a less intensively timed treatment regimen improved the outcome. Further studies are needed to define the optimal regimen for treating myeloid leukaemia of DS.

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Authors and Affiliations

  1. Department of Paediatrics, Skejby Hospital, 8200, Aarhus N, Denmark

    Lotte Abildgaard, Eva Ellebæk & Henrik Hasle

  2. Barncancerforskningsenheten, Astrid Lindgrens Barnsjukhus, Stockholm, Sweden

    Göran Gustafsson

  3. Department of Paediatrics, Queen Silvia Children’s Hospital, Gothenburg, Sweden

    Jonas Abrahamsson

  4. Department of Paediatrics, University of Helsinki, Helsinki, Finland

    Liisa Hovi

  5. Department of Paediatrics, Landspitalinn, Reykjavik, Iceland

    Gudmundur Jonmundsson

  6. Department of Paediatrics, University Hospital, Rikshospitalet, Oslo, Norway

    Bernward Zeller

Authors
  1. Lotte Abildgaard
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  2. Eva Ellebæk
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  3. Göran Gustafsson
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  4. Jonas Abrahamsson
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  5. Liisa Hovi
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  6. Gudmundur Jonmundsson
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  7. Bernward Zeller
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  8. Henrik Hasle
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Correspondence to Henrik Hasle.

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Abildgaard, L., Ellebæk, E., Gustafsson, G. et al. Optimal treatment intensity in children with Down syndrome and myeloid leukaemia: data from 56 children treated on NOPHO-AML protocols and a review of the literature. Ann Hematol 85, 275–280 (2006). https://doi.org/10.1007/s00277-005-0045-5

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  • DOI: https://doi.org/10.1007/s00277-005-0045-5

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