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Myeloid neoplasm with prominent eosinophilia and PDGFRA rearrangement treated with imatinib mesylate
Authors:Mathias Rathe MD  Thomas Kielsgaard Kristensen PhD  Michael Boe Møller MD  DMSc  Niels LT Carlsen MD  DMSc
Institution:1. Department of Pediatric Hematology and Oncology, H.C. Andersen Children's Hospital, Odense University Hospital, Odense, Denmark;2. Department of Pathology, Odense University Hospital, Odense, Denmark
Abstract:The FIP1L1PDGFRA fusion gene is the most frequent genetic aberration in myeloid neoplasms associated with eosinophilia and abnormalities of PDGFRA, PDGFRB, or FGFR1. Affected patients in adult populations are very sensitive to imatinib therapy. Pediatric cases are rare and so far only one case of FIP1L1PDGFRA positive disease has been reported. We report a 2‐year‐old female with a myeloid neoplasm associated with eosinophilia and rearrangement of PDGFRA. Treatment with imatinib resulted in complete and durable clinical, hematological, and molecular remission within 3 months after starting treatment. Pediatr Blood Cancer. 2010;55:730–732. © 2010 Wiley‐Liss, Inc.
Keywords:eosinophilia  FIP1L1–  PDGFRA  pediatric hematology/oncology  tyrosine kinase  tyrosine kinase inhibitor
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