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Congenital and Childhood myeloproliferative disorders with eosinophilia responsive to imatinib
Author(s) -
Abraham Shirley,
Salama Mohamed,
Hancock Jeffrey,
Jacobsen Jeffrey,
Fluchel Mark
Publication year - 2012
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.24148
Subject(s) - pdgfrb , medicine , eosinophilia , imatinib , imatinib mesylate , pdgfra , myeloproliferative disorders , hypereosinophilic syndrome , polycythemia vera , oncology , gastroenterology , stromal cell , myeloid leukemia , gist , biochemistry , chemistry , gene
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. Pediatr Blood Cancer 2012; 59: 928–929. © 2012 Wiley Periodicals, Inc.