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lüll The FIP1L1-PDGFRalpha fusion tyrosine kinase in hypereosinophilic syndrome and chronic eosinophilic leukemia: implications for diagnosis, classification, and management Gotlib J; Cools J; Malone JM 3rd; Schrier SL; Gilliland DG; Coutre SEBlood 2004[Apr]; 103 (8): 2879-91Idiopathic hypereosinophilic syndrome (HES) and chronic eosinophilic leukemia (CEL) comprise a spectrum of indolent to aggressive diseases characterized by unexplained, persistent hypereosinophilia. These disorders have eluded a unique molecular explanation, and therapy has primarily been oriented toward palliation of symptoms related to organ involvement. Recent reports indicate that HES and CEL are imatinib-responsive malignancies, with rapid and complete hematologic remissions observed at lower doses than used in chronic myelogenous leukemia (CML). These BCR-ABL-negative cases lack activating mutations or abnormal fusions involving other known target genes of imatinib, implicating a novel tyrosine kinase in their pathogenesis. A bedside-to-benchtop translational research effort led to the identification of a constitutively activated fusion tyrosine kinase on chromosome 4q12, derived from an interstitial deletion, that fuses the platelet-derived growth factor receptor-alpha gene (PDGFRA) to an uncharacterized human gene FIP1-like-1 (FIP1L1). However, not all HES and CEL patients respond to imatinib, suggesting disease heterogeneity. Furthermore, approximately 40% of responding patients lack the FIP1L1-PDGFRA fusion, suggesting genetic heterogeneity. This review examines the current state of knowledge of HES and CEL and the implications of the FIP1L1-PDGFRA discovery on their diagnosis, classification, and management.|Algorithms[MESH]|Antineoplastic Agents/therapeutic use[MESH]|Benzamides[MESH]|Cytogenetics[MESH]|Humans[MESH]|Hypereosinophilic Syndrome/classification/diagnosis/drug therapy/*genetics[MESH]|Imatinib Mesylate[MESH]|Oncogene Proteins, Fusion[MESH]|Piperazines/therapeutic use[MESH]|Prognosis[MESH]|Protein-Tyrosine Kinases/*genetics[MESH]|Pyrimidines/therapeutic use[MESH]|Receptor, Platelet-Derived Growth Factor alpha/*genetics[MESH]|T-Lymphocytes/immunology[MESH]|mRNA Cleavage and Polyadenylation Factors/*genetics[MESH] |