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Deletion of 11q23 is a highly specific nonrandom secondary genetic abnormality of ETV6/RUNX1-rearranged childhood acute lymphoblastic leukemia

Leukemia volume 21, pages 584–586 (2007)Cite this article

An Erratum to this article was published on 25 April 2007

The term ‘11q23 abnormalities’ was originally used to describe various morphological changes of the terminal part of chromosome 11q that are evident on the cytogenetic level only. However, following the adoption of this term by molecular biologists and clinicians, it is now used to denote all genetic alterations that are encountered within this region regardless of the method of their ascertainment. Chromosome band 11q23 is the site of the mixed lineage leukemia (MLL) gene that is most often disrupted in 11q23 abnormalities of various hematological neoplasms. Although 11q23/MLL aberrations are usually recognized as being reciprocal on the chromosomal level, few could nevertheless be interpreted as an unbalanced rearrangement or deletion. Thus, apart from its value to detect virtually all MLL-involving rearrangements on a single-cell level, dual-color fluorescence in situ hybridization (FISH) analysis with 3′ and 5′ MLL split-apart probes is also very helpful to discover cases with either 3′ MLL or even complete MLL deletions.1

Genetic abnormalities involving the 11q23/MLL region account for about 5–10% of childhood acute lymphoblastic leukemia (ALL). Patients with an 11q23 deletion in which an entire MLL allele may or may not be eliminated account for approximately 20% of the 11q23-rearranged cases.2, 3 Although there are limited data on the biological and clinical characteristics as well as the prognosis of a del(11)(q23) in pediatric ALL, a few previous studies showed that this abnormality was commonly associated with low-risk features at presentation and survival rates of approximately 75%.2, 3 The detection of several cases with such 11q deletions during our screening program for secondary genetic abnormalities in ETV/RUNX1+ B-cell precursor (BCP) ALL led us assess the incidence as well as the clinical, laboratory and biological features of 11q23-deleted cases in a population-based cohort of consecutively registered BCP ALL patients.4

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Acknowledgements

This work was supported by the research program Genome Research for Health of the Austrian Ministry of Education, Science and Culture (GEN-AU Child, GZ 200.071/3-VI/2a/2002), and the Fonds zur Förderung der wissenschaftlichen Forschung (Grant P15150) to OAH.

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  1. Department of Pediatric Hematology and Oncology, St Anna Children's Hospital, Vienna, Austria

    A Attarbaschi, G Mann, M Steiner, M N Dworzak, H Gadner & O A Haas

  2. Children's Cancer Research Institute (CCRI), St Anna Children's Hospital, Vienna, Austria

    S Strehl, M König, V Jeitler, Th Lion, M N Dworzak, H Gadner & O A Haas

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  1. A Attarbaschi
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on behalf of the Austrian Berlin-Frankfurt–Münster (BFM) Cooperative Study Group

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Correspondence to A Attarbaschi.

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Attarbaschi, A., Mann, G., Strehl, S. et al. Deletion of 11q23 is a highly specific nonrandom secondary genetic abnormality of ETV6/RUNX1-rearranged childhood acute lymphoblastic leukemia. Leukemia 21, 584–586 (2007). https://doi.org/10.1038/sj.leu.2404507

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