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The clinical relevance of BAALC and ERG expression levels in pediatric AML

Leukemia volume 27, pages 735–737 (2013)Cite this article

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Pediatric acute myeloid leukemia (AML) is a heterogeneous disease,1 for which survival rates have increased up to 70% over the past years.2 Approximately 45% of adult AML patients and 20% of pediatric AML patients are diagnosed with cytogenetically normal AML (CN-AML), which is currently classified and treated as a homogeneous intermediate-risk group.3 For future stratification of pediatric CN-AML, it is important to unravel the underlying molecular aberrations. For example, single-gene mutations such as NPM1 and CEBPA double mutations appeared to predict favorable outcome, whereas WT1 mutations and the cryptic NUP98/NSD1 translocation4 are of poor prognostic significance. In adult AML patients, the expression levels of specific genes have been associated with prognosis, mainly in CN-AML. For instance, high expression levels of BAALC (brain and acute leukemia cytoplasmic; 8q22) or ERG (E26 transformation-specific-related; 21q22) genes predict a poor prognosis in most adult AML studies, confined to CN-AML, as described by Schwind et al.5

However, in two pediatric studies on this subject so far, conflicting results emerged. The NOPHO group confirmed that high BAALC expression was an independent prognostic factor for event-free survival (EFS), which, however, did not lead to an overall survival (OS) benefit. ERG expression had no prognostic impact and, in fact, only high-risk cytogenetics predicted for OS in multivariate analysis.6 In the study by Pigazzi et al., independent prognostic significance of high ERG expression was found in 71 mixed-lineage leukemia (MLL)-rearranged patients, but not when analyzing all patients together, as was later confirmed by the NOPHO group.6, 7, 8

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Acknowledgements

MCHH was funded by KIKA, project number 64, entitled: Aberrant signal transduction profiling in pediatric AML.

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Authors and Affiliations

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

    M C H Hermkens, M M van den Heuvel-Eibrink, S T C J M Arentsen-Peters, R Pieters & C M Zwaan

  2. Department of Haematology, CHU de Paris - Hôpital Robert Debré, Paris, France

    A Baruchel

  3. Department of Pediatric Hematology/Oncology, 2nd Medical School, Charles University, Prague, Czech Republic

    J Stary

  4. Department of Pediatric Oncology/Hematology, AML-BFM Study Group, Medical High School, Hannover, Germany

    D Reinhardt & M Zimmerman

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

    V de Haas

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  1. M C H Hermkens
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Correspondence to C M Zwaan.

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Hermkens, M., van den Heuvel-Eibrink, M., Arentsen-Peters, S. et al. The clinical relevance of BAALC and ERG expression levels in pediatric AML. Leukemia 27, 735–737 (2013). https://doi.org/10.1038/leu.2012.233

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