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Chronic Myeloproliferative Disorders

WT1 transcript amount discriminates secondary or reactive eosinophilia from idiopathic hypereosinophilic syndrome or chronic eosinophilic leukemia

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Abstract

Idiopathic hypereosinophilic syndromes (HES) comprise a spectrum of indolent to aggressive diseases characterized by persistent hypereosinophilia. Hypereosinophilia can result from the presence of a defect in the hematopoietic stem cell giving rise to eosinophilia, it can be present in many myeloproliferative disorders or alternatively it may be a reactive form, secondary to many clinical conditions. The hybrid gene FIP1L1-PDGRFα was identified in a subset of patients presenting with HES or chronic eosinophilic leukemia (CEL). In spite of this, the majority of HES patients do not present detectable molecular lesions and for many of them the diagnosis is based on exclusion criteria and sometimes it remains doubt. In this study we explored the possibility to distinguish between HES/CEL and reactive hypereosinophilia based on WT1 transcript amount. For this purpose, 312 patients with hypereosinophilia were characterized at the molecular and cytogenetic level and analyzed for WT1 expression at diagnosis and during follow-up. This study clearly demonstrates that WT1 quantitative assessment allows to discriminate between HES/CEL and reactive eosinophilia and represents a useful tool for disease monitoring especially in the patients lacking a marker of clonality.

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Acknowledgements

This work was supported by grants from AIRC (Associazione Italiana per la Ricerca sul Cancro), MURST-COFIN, AIL (Associazione Italiana contro le Leucemie) and Regione Piemonte.

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Authors and Affiliations

  1. Division of Hematology and Internal Medicine, Department of Clinical and Biological Sciences, University of Turin, Turin, Italy

    D Cilloni, F Messa, E Gottardi, F Arruga, I Defilippi, S Carturan, E Messa, M Fava, E Giugliano, V Rosso, R Catalano, P Nicoli & G Saglio

  2. Department of Hematology, Seragnoli Institute, University of Bologna, Bologna, Italy

    G Martinelli, M Rondoni, E Ottaviani, S Soverini & M Baccarani

  3. Department of Hematology, University of Pavia, Pavia, Italy

    S Merante

  4. Department of Hematology, University of Udine, Udine, Italy

    M Tiribelli

  5. Department of Hematology, University of Naples, Naples, Italy

    F Pane

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  1. D Cilloni
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  2. F Messa
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  3. G Martinelli
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  4. E Gottardi
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  5. F Arruga
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  6. I Defilippi
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  7. S Carturan
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  8. E Messa
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  9. M Fava
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  10. E Giugliano
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  11. V Rosso
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  12. R Catalano
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  13. S Merante
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  14. P Nicoli
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  15. M Rondoni
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  16. E Ottaviani
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  17. S Soverini
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  18. M Tiribelli
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  19. F Pane
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  20. M Baccarani
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  21. G Saglio
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Consortia

a study by the GIMEMA Working Party on Chronic Myeloid Leukemia

Corresponding author

Correspondence to D Cilloni.

Appendix

Appendix

The following authors have actively participated in this study: Anna Serra, Enrico Bracco, Alessandro Morotti (Torino); Francesco Onida (Milano); Alessandro Levis, Massimo Pini (Alessandria); Tomasz Sacha (Cracow); Francesco Iuliano (Catanzaro); Marina Liberati (Perugia); Francesco Buccisano (Roma); Alberto Santagostino (Vercelli); Lucia Tornagli (Monza); Gianluca Gaidano (Novara); Gianpaolo Fra (Novara); Dario Ferrero (Torino); Massimo Negrini (Ferrara); Mariano Rocchi (Bari).

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Cilloni, D., Messa, F., Martinelli, G. et al. WT1 transcript amount discriminates secondary or reactive eosinophilia from idiopathic hypereosinophilic syndrome or chronic eosinophilic leukemia. Leukemia 21, 1442–1450 (2007). https://doi.org/10.1038/sj.leu.2404670

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  • DOI: https://doi.org/10.1038/sj.leu.2404670

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