title: [Idiopathic hypereosinophilic syndrome: toward a new molecular-targeted therapy and a new cytomorphological and molecular definition].
abstract: Idiopathic hypereosinophilic syndrome is characterised by chronic hypereosinophilia leading to tissue damage, and after exclusion of reactive eosinophilia. Until recently no specific or efficient therapeutic was available. In 2003, a recurrent interstitial deletion 4q12 leading to the fusion of the FIP1L1 and PDGFRA genes was detected in hypereosinophilic syndromes. The resulting protein has constitutive tyrosine kinase activity which explains clinical and cytological remission of hypereosinophilic syndrome after treatment by a specific tyrosine kinase inhibitor, imatinib mesylate or Glivec, usually used in chronic myeloid leukaemia. Here we report a patient with hypereosinophilic syndrome associated to peculiar morphology of neutrophilic series and the 4q12 deletion. He presented clinical and haematological remission since the introduction of imatinib mesylate therapy.
authors.affiliation: Laboratoire d'hématologie biologique, UPRES EA-3863, Centre hospitalo-universitaire, Angers. OdBlanchet@chu-angers.fr
authors.forename: M-P
authors.initials: MP
authors.lastname: Moles
authors.forename: J
authors.initials: J
authors.lastname: Landry
authors.forename: C
authors.initials: C
authors.lastname: Roche-Lestienne
authors.forename: A
authors.initials: A
authors.lastname: Godon
authors.forename: A
authors.initials: A
authors.lastname: Schmidt-Tanguy
authors.forename: M
authors.initials: M
authors.lastname: Gardembas
authors.forename: C
authors.initials: C
authors.lastname: Le Clech
authors.forename: J-L
authors.initials: JL
authors.lastname: Verret
authors.forename: M
authors.initials: M
authors.lastname: Zandecki
authors.forename: O
authors.initials: O
authors.lastname: Blanchet
mesh_terms: D000328:Adult; D001549:Benzamides; D006801:Humans; D017681:Hypereosinophilic Syndrome / Q000188:drug therapy* / Q000209:etiology / Q000235:genetics / Q000473:pathology*; D000068877:Imatinib Mesylate; D008297:Male; D010879:Piperazines / Q000627:therapeutic use*; D047428:Protein Kinase Inhibitors / Q000627:therapeutic use*; D011743:Pyrimidines / Q000627:therapeutic use*
chemical_list: D001549:Benzamides; D010879:Piperazines; D047428:Protein Kinase Inhibitors; D011743:Pyrimidines; D000068877:Imatinib Mesylate