title: [New insights into hypereosinophilic syndromes].
abstract: Hypereosinophilic syndrome (HES) is characterized by chronic unexplained eosinophilia with organ involvement. The concept of HES as a single disease entity is being challenged by the recent identification of multiple underlying molecular mechanisms. HES can directly affect the eosinophil lineage (often linked to a fusion gene FIP1L1-PDGFRA, and corresponding in this case to chronic eosinophilic leukemia), or the lymphoid lineage, where eosinophilia is secondary to expansion of a T cell subset overproducing interleukin-5, a cytokine involved in eosinophilopoiesis. These recent discoveries have legitimized the use of tyrosine kinase inhibitors such as imatinib, which, by inhibiting PDGFRA, have transformed the prognosis of chronic eosinophilic leukemia, and also the use of monoclonal anti-IL-5 antibodies, which are promising treatment for steroid-dependent HES.
authors.affiliation: Hôpital Foch, 40, rue Worth, 92151 Suresne cedex,
authors.forename: Olivier
authors.initials: O
authors.lastname: Bletry
authors.forename: Jean-Emmanuel
authors.initials: JE
authors.lastname: Kahn
authors.forename: Felix
authors.initials: F
authors.lastname: Ackermann
authors.forename: Pierre
authors.initials: P
authors.lastname: Charles
authors.forename: Fanny
authors.initials: F
authors.lastname: Legrand
mesh_terms: D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D061067:Antibodies, Monoclonal, Humanized; D016207:Cytokines / Q000378:metabolism; D006801:Humans; D017681:Hypereosinophilic Syndrome / Q000188:drug therapy* / Q000235:genetics*; D015848:Interleukin-5 / Q000037:antagonists & inhibitors; D015514:Oncogene Proteins, Fusion / Q000235:genetics; D047428:Protein Kinase Inhibitors / Q000627:therapeutic use; D020796:Receptor, Platelet-Derived Growth Factor alpha / Q000235:genetics; D016176:T-Lymphocyte Subsets / Q000276:immunology; D039221:mRNA Cleavage and Polyadenylation Factors / Q000235:genetics
chemical_list: D000911:Antibodies, Monoclonal; D061067:Antibodies, Monoclonal, Humanized; D016207:Cytokines; D015848:Interleukin-5; D015514:Oncogene Proteins, Fusion; D047428:Protein Kinase Inhibitors; D039221:mRNA Cleavage and Polyadenylation Factors; C434107:mepolizumab; C479819:FIP1L1-PDGFRA fusion protein, human; D020796:Receptor, Platelet-Derived Growth Factor alpha