WT1 transcript amount discriminates secondary or reactive eosinophilia from idiopathic hypereosinophilic syndrome or chronic eosinophilic leukemia
- 17 May 2007
- journal article
- Published by Springer Science and Business Media LLC in Leukemia
- Vol. 21 (7), 1442-1450
- https://doi.org/10.1038/sj.leu.2404670
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.Keywords
This publication has 29 references indexed in Scilit:
- FIP1L1-PDGFRA fusion: prevalence and clinicopathologic correlates in 89 consecutive patients with moderate to severe eosinophiliaBlood, 2004
- The FIP1L1-PDGFRα fusion tyrosine kinase in hypereosinophilic syndrome and chronic eosinophilic leukemia: implications for diagnosis, classification, and managementBlood, 2004
- A Tyrosine Kinase Created by Fusion of thePDGFRAandFIP1L1Genes as a Therapeutic Target of Imatinib in Idiopathic Hypereosinophilic SyndromeNew England Journal of Medicine, 2003
- The Hypereosinophilic Syndrome RevisitedAnnual Review of Medicine, 2003
- Treatment of hypereosinophilic syndrome with imatinib mesilateThe Lancet, 2002
- HypereosinophiliaCurrent Opinion in Hematology, 2000
- Abnormal Clones of T Cells Producing Interleukin-5 in Idiopathic EosinophiliaNew England Journal of Medicine, 1999
- Aberrant Cytokine Production By Sezary Syndrome Patients: Cytokine Secretion Pattern Resembles Murine Th2 CellsJournal of Investigative Dermatology, 1992
- Isolation and characterization of a zinc finger polypeptide gene at the human chromosome 11 Wilms' tumor locusCell, 1990
- The Hypereosinophilic SyndromesAnnals of Internal Medicine, 1968