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Acute Leukemias

Long-term results of NOPHO ALL-92 and ALL-2000 studies of childhood acute lymphoblastic leukemia

Leukemia volume 24, pages 345–354 (2010)Cite this article

A Corrigendum to this article was published on 10 March 2010

Abstract

Analysis of 2668 children with acute lymphoblastic leukemia (ALL) treated in two successive Nordic clinical trials (Nordic Society of Paediatric Haematology and Oncology (NOPHO) ALL-92 and ALL-2000) showed that 75% of all patients are cured by first-line therapy, and 83% are long-term survivors. Improvements in systemic and intrathecal chemotherapy have reduced the use of central nervous system (CNS) irradiation to <10% of the patients and provided a 5-year risk of isolated CNS relapse of 2.6%. Improved risk stratification and chemotherapy have eliminated the previous independent prognostic significance of gender, CNS leukemia and translocation t(1;19)(q23;p13), whereas the post-induction level of minimal residual disease (MRD) has emerged as a new risk grouping feature. Infant leukemia, high leukocyte count, T-lineage immunophenotype, translocation t(4;11)(q21;q23) and hypodiploidy persist to be associated with lower cure rates. To reduce the overall toxicity of the treatment, including the risk of therapy-related second malignant neoplasms, the current NOPHO ALL-2008 protocol does not include CNS irradiation in first remission, the dose of 6-mercaptopurine is reduced for patients with low thiopurine methyltransferase activity, and the protocol restricts the use of hematopoietic stem cell transplantation in first remission to patients without morphological remission after induction therapy or with high levels of MRD after 3 months of therapy.

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Acknowledgements

This work was supported by The Childhood Cancer Foundation, Denmark; The Childhood Cancer Foundation, Sweden; The University Hospital Rigshospitalet; Michael Goldschmidt Holding A/S; the Nona and Kullervo Väre Foundation. Kjeld Schmiegelow holds the Childhood Cancer Foundation Research Professorship in Pediatric Oncology.

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Authors and Affiliations

  1. The Faculty of Medicine, The Institute of Gynaecology, Obstetrics, and Paediatrics, University of Copenhagen, Copenhagen, Denmark

    K Schmiegelow

  2. Department of Pediatrics, University Hospital Rigshospitalet, Copenhagen, Denmark

    K Schmiegelow

  3. Department of Pediatrics, University Hospital, Umeå, Sweden

    E Forestier

  4. Department of Pediatrics, Ullevål Hospital, Oslo, Norway

    M Hellebostad

  5. Department of Pediatric Oncology, Astrid Lindgrens Childrens Hospital, Stockholm, Sweden

    M Heyman & S Söderhäll

  6. Department of Pediatrics, University Hospital Landspitalin, Reykjavik, Iceland

    J Kristinsson

  7. Department of Pediatrics, University Hospital, Helsinki, Finland

    M Taskinen

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Correspondence to K Schmiegelow.

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Schmiegelow, K., Forestier, E., Hellebostad, M. et al. Long-term results of NOPHO ALL-92 and ALL-2000 studies of childhood acute lymphoblastic leukemia. Leukemia 24, 345–354 (2010). https://doi.org/10.1038/leu.2009.251

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