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Deletion of the inositide-specific phospholipase c β1 gene is a rare event in myelodysplastic syndrome and thus of limited value to predict leukemic progression

Leukemia volume 19, pages 2011–2013 (2005)Cite this article

TO THE EDITOR

The myelodysplastic syndromes (MDS) are a heterogeneous group of hematological disorders displaying the common characteristics of peripheral blood cytopenias, bone marrow (BM) (hyper)activity and the variable occurrence of karyotypic and leukemic evolution. MDS affects mainly older individuals and in some 30–50% of patients karyotypic aberrations are found. Leukemic progression occurs in about a third of cases and is a major cause of death. Specific karyotypic aberrations are known to be associated with poor outcome and leukemic transformation. The refined cytogenetic stratification of the International Prognostic Scoring System (IPSS) enabled clinicians to better predict disease evolution than was previously possible in the subgroups of the French-American-British (FAB) classification.1, 2 However, especially in the low and intermediate-1 risk IPSS categories, where mostly normal or low-risk karyotypic changes are found, the outcome remains highly variable.3 This has prompted investigators to search for disease characteristics which better predict for progressive disease. Multilineage dysplasia is associated with inferior outcome and has been incorporated into the new World Health Organization classification for MDS.4 In addition, we have showed that histological parameters such as abnormal localization of immature myeloid precursors and increased CD34 immunostaining can effectively discriminate patients at risk for leukemic evolution and poor outcome in the subgroups of the IPSS.3

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Acknowledgements

This text presents research results of the Belgian programme on Interuniversity Poles of Attraction initiated by the Belgian State, Prime Minister's Office, Science Policy Programming. The scientific responsibility is assumed by the authors.

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Authors and Affiliations

  1. Department of Hematology, University Hospitals, University of Leuven, Leuven, Belgium

    E Verburgh & G Verhoef

  2. Center for Human Genetics, University of Leuven, Leuven, Belgium

    P Vandenberghe & A Hagemeijer

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  1. E Verburgh
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  2. G Verhoef
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  3. P Vandenberghe
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  4. A Hagemeijer
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Correspondence to A Hagemeijer.

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Verburgh, E., Verhoef, G., Vandenberghe, P. et al. Deletion of the inositide-specific phospholipase c β1 gene is a rare event in myelodysplastic syndrome and thus of limited value to predict leukemic progression. Leukemia 19, 2011–2013 (2005). https://doi.org/10.1038/sj.leu.2403964

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