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BTG1 deletions do not predict outcome in Down syndrome acute lymphoblastic leukemia

Leukemia volume 27, pages 251–252 (2013)Cite this article

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Children with Down syndrome (DS) have an increased risk of developing acute myeloid and B-cell precursor acute lymphoblastic leukemia (BCP-ALL).1 The prognosis of DS ALL is at best similar and often inferior to that of sporadic ALL (non-DS) patients.2, 3 DS ALL is characterized by unique biological features when compared with non-DS ALL. For instance, DS ALL has a lower frequency of the favorable genetic abnormalities t(12;21)(p13;q22) (ETV6–RUNX1) and the unfavorable t(9;22)(q34;q11) (BCR-ABL1),3, 4 but a higher frequency of JAK2 mutations and CRLF2 rearrangements.5, 6 Using genome-wide screening techniques, several (novel) genomic aberrations involved in the pathogenesis of (non-) DS ALL were identified.7, 8 The potential prognostic impact of most of these novel aberrations and whether these patients may benefit from specific therapies targeted to these unique genetic features needs to be investigated further.

The B-cell translocation gene 1 (BTG1) was recently described as a recurrent lesion in pediatric BCP-ALL.7, 9, 10 It was originally identified as a translocation partner of c-MYC in B-cell chronic lymphocytic leukemia.11, 12 BTG1 is a highly conserved gene and belongs to the family of BTG/TOB genes.11, 13 It has a role in several crucial cellular processes, such as proliferation, and apoptosis. Recently, Lundin et al.14 reported on a high frequency of BTG1 deletions (∼29%) in DS ALL. However, the study included a limited number of DS ALL patients (n=17) and hence may not be an accurate estimate of the frequency of BTG1 deletions in DS ALL. Therefore, we investigated the frequency of BTG1 deletions in a large series of DS ALL patients and in addition analyzed the prognostic significance of BTG1 abnormalities.

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Acknowledgements

This study was supported by research funding from the foundation Kinderen Kankervrij (KIKA) to TDB and CMZ and the Leukemia and Lymphoma Research (LLR) to CS, CJH and AVM.

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Authors and Affiliations

  1. Pediatric Oncology/Hematology, Erasmus MC–Sophia Children’s Hospital, Rotterdam, The Netherlands

    T D Buitenkamp, R Pieters, M M van den Heuvel-Eibrink & C M Zwaan

  2. Dutch Childhood Oncology Group, Den Haag, The Netherlands

    R Pieters & V de Haas

  3. Acute Myeloid Leukemia-Berlin-Frankfurt-Münster Study Group, Pediatric -Hematology/Oncology, Medical School Hannover, Hannover, Germany

    M Zimmermann

  4. Clinical Trial Service Unit, University of Oxford, Oxford, UK

    S M Richards

  5. Department of Haematology, Sheffield Children’s Hospital, Sheffield, UK

    A J Vora

  6. Department of Paediatric Haematology/Oncology, John Radcliffe Hospital, Oxford, UK

    C D Mitchell

  7. Leukaemia Research Cytogenetics Group, Northern Institute for Cancer Research, Newcastle University, Newcastle, UK

    C Schwab, C J Harrison & A V Moorman

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  1. T D Buitenkamp
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Correspondence to C M Zwaan.

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Buitenkamp, T., Pieters, R., Zimmermann, M. et al. BTG1 deletions do not predict outcome in Down syndrome acute lymphoblastic leukemia. Leukemia 27, 251–252 (2013). https://doi.org/10.1038/leu.2012.199

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