1. ¹Division of Hematology and Internal Medicine, Department of Clinical and Biological Sciences, University of Turin, Turin, Italy
2. ²Department of Hematology, Seragnoli Institute, University of Bologna, Bologna, Italy
3. ³Department of Hematology, University of Pavia, Pavia, Italy
4. ⁴Department of Hematology, University of Udine, Udine, Italy
5. ⁵Department of Hematology, University of Naples, Naples, Italy

Correspondence: Dr D Cilloni, Department of Clinical and Biological Sciences, University of Turin, San Luigi Hospital, Gonzole 10, 10043 Orbassano-Torino, Italy. E-mail: daniela.cilloni@unito.it

Received 3 January 2006; Revised 20 January 2007; Accepted 19 February 2007; Published online 17 May 2007.

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-PDGRF 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.