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ERG deletion is associated with CD2 and attenuates the negative impact of IKZF1 deletion in childhood acute lymphoblastic leukemia

Leukemia volume 28, pages 182–185 (2014)Cite this article

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A Corrigendum to this article was published on 06 May 2015

Childhood acute lymphoblastic leukemia (ALL) demonstrates clinical and biological heterogeneity, which is largely sustained by a diverse genetic background.1, 2, 3 Today, the majority of children with ALL treated on modern treatment protocols can expect to achieve long-term cure, but still a significant proportion of patients suffer therapy-related toxicity and disease relapse.2, 3 Thus, identification of new prognostic markers to improve initial risk assessment for subsequent treatment tailoring remains an important goal in ALL research.

Recent genomic profiling studies of childhood ALL identified several new recurrent structural genetic aberrations including monoallelic intragenic deletions of ERG (ERGdel)—a gene coding for an ETS family member transcription factor with important functions in hematopoiesis—in approximately 7% of childhood B-cell precursor (BCP) ALL.1, 2, 4 ERGdel was later associated with a specific gene expression profile including aberrant expression of CD2 and suggested to characterize a novel ALL subgroup with specific biology and clinical behavior.5, 6 Of importance, in previous studies on high-risk BCP ALL, ERGdel-positive cases could be classified into a gene expression cluster with significantly better treatment outcome.7, 8 The above findings suggest that ERGdel may be a clinically useful marker to further refine risk-adapted treatment stratification in childhood ALL.

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Acknowledgements

We thank all participants and personnel involved in ALL-BFM 2000 and Ester Mejstrikova for sharing her expertise in flow cytometric assessment of CD2-positivity. This work was supported by EU FP7 (ENCCA), the Madeleine Schickedanz-Kinderkrebsstiftung, Deutsche Krebshilfe, GACR-P302/12/G101, UNCE204012, MZCR-00064203.

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Authors and Affiliations

  1. Department of Pediatrics, University Hospital Schleswig-Holstein, Kiel, Germany

    M Zaliova, P Dörge, A Möricke, B Meissner, M Schrappe & G Cario

  2. Department of Pediatric Hematology and Oncology, CLIP—Childhood Leukemia Investigation Prague, 2nd Faculty of Medicine, Charles University and University Hospital Motol, Prague, Czech Republic

    M Zaliova, O Zimmermannova, J Stuchly & J Zuna

  3. Pediatric Hematology and Oncology, Charité University Hospital, Berlin, Germany

    C Eckert, L Karawajew & P Rhein

  4. Pediatric Hematology and Oncology, Hannover Medical School, Hannover, Germany

    M Zaliova, M Zimmermann & M Stanulla

  5. Oncogenetic laboratory, Pediatric Hematology and Oncology, University of Giessen, Giessen, Germany

    A Teigler-Schlegel

  6. Department of Human Genetics, University of Heidelberg, Heidelberg, Germany

    R Koehler & C R Bartram

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  1. M Zaliova
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Correspondence to M Stanulla.

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The authors declare no conflict of interest.

Additional information

MZ, GC and MST designed the study; MZ, OZ, BM and PD performed laboratory work; MZ, JS and MZI performed data analysis; CE performed analyses on relapse samples; AM and MS contributed to data interpretation and co-ordinate the ALL-BFM study group (chair: MS); RK and CRB performed MRD analyses; ATS performed cytogenetic and molecular genetic investigations; LK and PR performed immunophenotyping; MZ, JZ and MST wrote the manuscript; all authors criticially evaluated the manuscript and approved its content.

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Zaliova, M., Zimmermannova, O., Dörge, P. et al. ERG deletion is associated with CD2 and attenuates the negative impact of IKZF1 deletion in childhood acute lymphoblastic leukemia. Leukemia 28, 182–185 (2014). https://doi.org/10.1038/leu.2013.282

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