%0 Journal Article
%T Current Concepts on the Pathogenesis of the Hypereosinophilic Syndrome/Chronic Eosinophilic Leukemia
%A Yoshiyuki Yamada
%A Marc E. Rothenberg and Jose A. Cancelas
%J Translational Oncogenomics
%D 2012
%I 
%X Chronic eosinophilic leukemia is a clonal disease characterized by hypereosinophilia and eosinophilia-related pathologic manifestations. Recently, the fusion gene FIP1L1/PDGFRA was found in the long arm of chromosome 4 and its expression has been shown to be associated with development of a clinical hypereosinophilic syndrome (HES) in a significant proportion of patients. FIP1L1/PDGFR¦Á, the product of the gene FIP1L1/PDGFRA, is a constitutively activated tyrosine kinase and can be inhibited by imatinib mesylate. Several investigations have tried to dissect the mechanism of leukemogenesis and signaling induced by FIP1L1/PDGFR¦Á in cell lines, primary human eosinophils and in murine myeloproliferative models. In this review, we analyzed the current knowledge on the relationship between FIP1L1/PDGFR¦Á-induced signaling and eosinophil proliferation, survival and activation, specially focusing on its possible role in the modulation of cytokine and chemoattractant signaling pathways.
%U http://www.la-press.com/current-concepts-on-the-pathogenesis-of-the-hypereosinophilic-syndrome-article-a167