title: WT1 transcript amount discriminates secondary or reactive eosinophilia from idiopathic hypereosinophilic syndrome or chronic eosinophilic leukemia.
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-PDGRFalpha 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.
authors.affiliation: Division of Hematology and Internal Medicine, Department of Clinical and Biological Sciences, University of Turin, Turin, Italy. daniela.cilloni@unito.it
authors.forename: D
authors.initials: D
authors.lastname: Cilloni
authors.forename: F
authors.initials: F
authors.lastname: Messa
authors.forename: G
authors.initials: G
authors.lastname: Martinelli
authors.forename: E
authors.initials: E
authors.lastname: Gottardi
authors.forename: F
authors.initials: F
authors.lastname: Arruga
authors.forename: I
authors.initials: I
authors.lastname: Defilippi
authors.forename: S
authors.initials: S
authors.lastname: Carturan
authors.forename: E
authors.initials: E
authors.lastname: Messa
authors.forename: M
authors.initials: M
authors.lastname: Fava
authors.forename: E
authors.initials: E
authors.lastname: Giugliano
authors.forename: V
authors.initials: V
authors.lastname: Rosso
authors.forename: R
authors.initials: R
authors.lastname: Catalano
authors.forename: S
authors.initials: S
authors.lastname: Merante
authors.forename: P
authors.initials: P
authors.lastname: Nicoli
authors.forename: M
authors.initials: M
authors.lastname: Rondoni
authors.forename: E
authors.initials: E
authors.lastname: Ottaviani
authors.forename: S
authors.initials: S
authors.lastname: Soverini
authors.forename: M
authors.initials: M
authors.lastname: Tiribelli
authors.forename: F
authors.initials: F
authors.lastname: Pane
authors.forename: M
authors.initials: M
authors.lastname: Baccarani
authors.forename: G
authors.initials: G
authors.lastname: Saglio
mesh_terms: D000328:Adult; D000368:Aged; D002908:Chronic Disease; D003937:Diagnosis, Differential; D004802:Eosinophilia / Q000175:diagnosis*; D005260:Female; D006801:Humans; D017681:Hypereosinophilic Syndrome / Q000175:diagnosis*; D008297:Male; D008875:Middle Aged; D025202:Molecular Diagnostic Techniques; D012334:RNA, Neoplasm / Q000032:analysis*; D025721:WT1 Proteins / Q000032:analysis / Q000235:genetics*
chemical_list: D012334:RNA, Neoplasm; D025721:WT1 Proteins