title: FIP1L1-PDGFRα-positive hypereosinophilic syndrome in childhood: a case report and review of literature.
abstract: Hypereosinophilic syndromes in children are rare disorders traditionally characterized by an eosinophil count exceeding 1,500/mm³ on at least 2 occasions or evidence of tissue eosinophilia associated with symptoms and marked blood eosinophilia, lacking any secondary cause (such as infections, allergic disease, chemical-induced eosinophilia, hypoadrenalism, cancer). Until now there have only been 3 reported cases of pediatric FIP1L1-PDGFRα-positive hypereosinophilic syndromes. We describe a fourth patient, a white 14-year-old boy, the third treated with imatinib.
authors: *Pediatric Hematology and Oncology Unit, Oncology Department, A.R.N.A.S. Civico, Di Cristina and Benfratelli Hospitals ‡Hematology I, A.O. Villa Sofia-Cervello, Palermo †Molecular Biology Laboratory, Department of Clinical and Biologic Sciences, University of Turin, San Luigi Gonzaga Hospital, Orbassano, Turin, Italy.
authors: Piero
authors: P
authors: Farruggia
authors: Emilia
authors: E
authors: Giugliano
authors: Delia
authors: D
authors: Russo
authors: Angela
authors: A
authors: Trizzino
authors: Roberta
authors: R
authors: Lorenzatti
authors: Alessandra
authors: A
authors: Santoro
authors: Paolo
authors: P
authors: D'Angelo
mesh_terms: D000293:Adolescent; D017668:Age of Onset; D000970:Antineoplastic Agents / Q000627:therapeutic use; D001549:Benzamides / Q000627:therapeutic use; D001853:Bone Marrow / Q000473:pathology*; D006801:Humans; D017681:Hypereosinophilic Syndrome / Q000188:drug therapy / Q000235:genetics* / Q000473:pathology*; D000068877:Imatinib Mesylate; D008297:Male; D015514:Oncogene Proteins, Fusion / Q000235:genetics*; D010879:Piperazines / Q000627:therapeutic use; D011743:Pyrimidines / Q000627:therapeutic use; D020796:Receptor, Platelet-Derived Growth Factor alpha / Q000235:genetics*; D039221:mRNA Cleavage and Polyadenylation Factors / Q000235:genetics*
chemical_list: D000970:Antineoplastic Agents; D001549:Benzamides; D015514:Oncogene Proteins, Fusion; D010879:Piperazines; D011743:Pyrimidines; D039221:mRNA Cleavage and Polyadenylation Factors; D000068877:Imatinib Mesylate; C479819:FIP1L1-PDGFRA fusion protein, human; D020796:Receptor, Platelet-Derived Growth Factor alpha