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Dic(9;20)(p13;q11) in childhood acute lymphoblastic leukaemia is related to low cellular resistance to asparaginase, cytarabine and corticosteroids

Leukemia volume 23, pages 209–212 (2009)Cite this article

Dic(9;20)(p13;q11) was first described as a nonrandom chromosome abnormality in B-cell precursor acute lymphoblastic leukaemia (BCP ALL) in the mid 1990s,1, 2 and 71 dic(9;20)-positive cases have since then been reported.3, 4, 5 Approximately 90% of these cases were children or adolescents, with dic(9;20) occurring in about 2% of childhood BCP ALL.6 The recent review by Forestier et al.5 describes that dic(9;20)-leukaemias are of B-cell precursor immunophenotype, never have a high hyperdiploid modal number, show a female predominance, and have a significant age incidence peak at 3 years. Most patients are allocated to non-standard risk treatment arms due to high WBC (median 24 × 109/l) and a relatively high frequency of CNS disease or other extra-medullary leukaemia (EML) at diagnosis. The prognostic implications of dic(9;20) are to a large extent unknown. A relatively large proportion of the relapses reported in the literature have been extra-medullary, and post-relapse treatment including block therapy has been successful in several patients, as illustrated by a p-EFS of 0.62 and a predicted overall survival of 0.82 at 5 years for the 24 Nordic cases.5

The optimal treatment strategy for children with dic(9;20)-positive BCP ALL is not known. Test of cellular drug resistance in vitro might help to elucidate this problem, and we have therefore analysed samples from patients with and without dic(9;20) to see if this aberration is associated with a specific drug sensitivity profile. To our knowledge, no such data have been published so far.

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Acknowledgements

We thank Ms Anna-Karin Lannergård and Ms Annika Jonasson for skilful technical assistance, and all colleagues in the Nordic Society of Paediatric Haematology and Oncology who provided the patient samples. The Lions' Cancer Research Fund, the Swedish Child Cancer Foundation and the Nordic Cancer Union supported this work financially.

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Authors and Affiliations

  1. Department of Women's and Children's Health, University Children's Hospital, Uppsala, Sweden

    G Lönnerholm & B-M Frost

  2. Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden

    A Nordgren

  3. Department of Paediatrics, Landspitali University Hospital, Reykjavik, Iceland

    O G Jonsson

  4. Department of Hematology/Oncology and Stem Cell Transplantation, Hospital for Children and Adolescents, University of Helsinki, Helsinki, Finland

    J Kanerva

  5. Department of Paediatrics, St Olav University Hospital, Trondheim, Norway

    R Nygaard

  6. Paediatric Clinic II, Rigshospitalet, and the Medical Faculty, the University of Copenhagen, Copenhagen, Denmark

    K Schmiegelow

  7. Department of Medical Sciences, Section of Pharmacology, University Hospital, Uppsala, Sweden

    R Larsson

  8. Department of Clinical Sciences, Pediatrics, University of Umeå, Umeå, Sweden

    E Forestier

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  1. G Lönnerholm
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  2. A Nordgren
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  7. K Schmiegelow
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Correspondence to G Lönnerholm.

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Lönnerholm, G., Nordgren, A., Frost, BM. et al. Dic(9;20)(p13;q11) in childhood acute lymphoblastic leukaemia is related to low cellular resistance to asparaginase, cytarabine and corticosteroids. Leukemia 23, 209–212 (2009). https://doi.org/10.1038/leu.2008.179

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