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Unique BHLHB3 overexpression in pediatric acute myeloid leukemia with t(6;11)(q27;q23)

Leukemia volume 28, pages 1564–1568 (2014)Cite this article

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Within pediatric MLL-rearranged acute myeloid leukemia (AML), the translocation partners have been shown to be clinically highly relevant.1 The most striking example is the t(6;11)(q27;q23) subtype, which has an event-free survival of only 11% and estimated overall survival of only 22%.1 This poor outcome is determined by a high induction-failure rate (26%), as well as a high-relapse rate (57%).1

The fusion partner AF6/ MLLT4 is one of the atypical MLL-translocation partners, as the wildtype AF6 protein, in contrast to the majority of recurrent MLL-translocation partners, lacks presence and function in the MLL-associated chromatin-modifying protein complex.2

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Acknowledgements

This work was supported by the Rotterdam Oncology Research Foundation ‘KOCR’ (EAC) and the foundation KiKa, ‘Kinderen kankervrij’ (EAC, STA-P and RWS).

Author contributions

EAC, RP, CMZ and MMvdH-E conveyed and planned the study, collected and analyzed the data and wrote the paper; RWS participated in critical data review and in critical review and final approval of the paper; and JS, AB, VdH, DR, GJLK, CM, RM, LLN and MD participated in data and sample collection and in critical review and final approval of the paper.

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Authors and Affiliations

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

    E A Coenen, C M Zwaan, R W Stam, S T C J M Arentsen-Peters, R Pieters & M M van den Heuvel-Eibrink

  2. Dutch Childhood Oncology Group (DCOG), The Hague, The Netherlands

    C M Zwaan, V de Haas, R Pieters & M M van den Heuvel-Eibrink

  3. Pediatric Hematology/Oncology, Charles University and University Hospital Motol, Prague, Czech Republic

    J Stary

  4. Pediatric Hematology, Hôpital Robert Debré AP-HP and University ParisDiderot, Paris, France

    A Baruchel

  5. AML-BFM Study Group, Hannover, Germany

    D Reinhardt

  6. Pediatric Oncology/Hematology, VU University Medical Center, Amsterdam, The Netherlands

    G J L Kaspers

  7. Institute of Pharmaceutical Biology, ZAFES, Diagnostic Center of Acute Leukemia (DCAL), Frankfurt, Germany

    C Meyer & R Marschalek

  8. Center of Pediatric Hematology Oncology, Azienda Policlinico, Catania, Italy

    L L Nigro

  9. Department of Pediatrics, St Anna Children's Hospital and Children’s Cancer Research Institute, Medical University of Vienna, Vienna, Austria

    M Dworzak

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  1. E A Coenen
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  10. C Meyer
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  15. M M van den Heuvel-Eibrink
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Corresponding author

Correspondence to M M van den Heuvel-Eibrink.

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Coenen, E., Zwaan, C., Stary, J. et al. Unique BHLHB3 overexpression in pediatric acute myeloid leukemia with t(6;11)(q27;q23). Leukemia 28, 1564–1568 (2014). https://doi.org/10.1038/leu.2014.82

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