title: Congenital and childhood myeloproliferative disorders with eosinophilia responsive to imatinib.
abstract: Eosinophilia is seen in several myeloproliferative disorders (MPD). A subset of MPD involves the platelet-derived growth factor receptor beta (PDGFRB) gene. Imatinib mesylate has been efficacious in treating some of these MPDs. Here we describe two patients with MPD with eosinophilia and PDGFRB rearrangements, one of which was congenital. Both patients were treated with single agent imatinib and continue to be in clinical, hematologic, and cytogenetic remission despite weaning doses. No definite guidelines currently exist regarding the exact dosing and duration of imatinib therapy for these patients.
authors.affiliation: Pediatric Hematology Oncology, Department of Pediatrics, University of New Mexico, Albuquerque, New Mexico 87131-0001, USA. smabraham@salud.unm.edu
authors.forename: Shirley
authors.initials: S
authors.lastname: Abraham
authors.forename: Mohamed
authors.initials: M
authors.lastname: Salama
authors.forename: Jeffrey
authors.initials: J
authors.lastname: Hancock
authors.forename: Jeffrey
authors.initials: J
authors.lastname: Jacobsen
authors.forename: Mark
authors.initials: M
authors.lastname: Fluchel
mesh_terms: D000970:Antineoplastic Agents / Q000008:administration & dosage*; D001549:Benzamides; D002675:Child, Preschool; D004802:Eosinophilia / Q000150:complications / Q000188:drug therapy / Q000235:genetics / Q000473:pathology; D006801:Humans; D000068877:Imatinib Mesylate; D007231:Infant, Newborn; D007232:Infant, Newborn, Diseases / Q000188:drug therapy / Q000235:genetics / Q000473:pathology; D008297:Male; D009196:Myeloproliferative Disorders / Q000150:complications / Q000151:congenital / Q000188:drug therapy / Q000235:genetics / Q000473:pathology; D010879:Piperazines / Q000008:administration & dosage*; D011743:Pyrimidines / Q000008:administration & dosage*; D020797:Receptor, Platelet-Derived Growth Factor beta / Q000235:genetics*
chemical_list: D000970:Antineoplastic Agents; D001549:Benzamides; D010879:Piperazines; D011743:Pyrimidines; D000068877:Imatinib Mesylate; D020797:Receptor, Platelet-Derived Growth Factor beta