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Imatinib Treatment in PDGFRA‐Negative Childhood Hypereosinophilic Syndrome
Author(s) -
Weyand Angela C.,
Yanik Gregory A.,
Bailey Nathanael G.,
Wu YiMi,
Mody Rajen J.,
Castle Valerie P.
Publication year - 2016
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.25702
Subject(s) - medicine , hypereosinophilic syndrome , pdgfra , imatinib , imatinib mesylate , dermatology , cancer research , eosinophilia , gist , myeloid leukemia , stromal cell
We report a 4‐year‐old female who presented with severe hypereosinophilia (215.7 K/μl) and end‐organ dysfunction. Extensive evaluation including whole exome sequencing was performed, revealing no causative mutation. Initial treatment with corticosteroids, leukapheresis, and hydroxyurea decreased her absolute eosinophil count (AEC), although it remained elevated. Despite the absence of a PDGFRA mutation, an imatinib trial resulted in normalization of her AEC. Imatinib was discontinued after sustained normal counts for 1 month. AECs have remained normal for more than 1 year off therapy. This provides support for consideration of imatinib in the treatment of hypereosinophilia even in the absence of a known tyrosine kinase mutation. Pediatr Blood Cancer 2015; 9999:XX–XX © 2015 Wiley Periodicals, Inc.