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Myeloid neoplasm with prominent eosinophilia and PDGFRA rearrangement treated with imatinib mesylate
Author(s) -
Rathe Mathias,
Kristensen Thomas Kielsgaard,
Møller Michael Boe,
Carlsen Niels L.T.
Publication year - 2010
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.22655
Subject(s) - imatinib mesylate , medicine , pdgfra , eosinophilia , imatinib , neoplasm , cancer research , myeloid , mesylate , myeloproliferative neoplasm , myeloid leukemia , pathology , bone marrow , gist , stromal cell , myelofibrosis , chemistry , organic chemistry
The FIP1L1 – PDGFRA 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 FIP1L1 – PDGFRA 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.