Idiopathic hypereosinophilic syndrome presenting with severe vasculitis successfully treated with imatinib.

Content Provider	Europe PMC
Author	Fraticelli, Paolo Kafyeke, Alain Mattioli, Massimo Martino, Giuseppe Pio Murri, Marta Gabrielli, Armando
Copyright Year	2016
Abstract	Idiopathic hypereosinophilic syndrome (HES) is a rare disorder characterized by peripheral eosinophilia exceeding 1500/mm3, a chronic course, absence of secondary causes, and signs and symptoms of eosinophil-mediated tissue injury. One of the best-characterized forms of HES is the one associated with FIP1L1-PDGFRA gene rearrangement, which was recently demonstrated as responsive to treatment with the small molecule kinase inhibitor drug, imatinib mesylate. Here, we describe the case of a 51-year-old male, whose symptoms satisfied the clinical criteria for HES with cutaneous and cardiac involvement and who also presented with vasculitic brain lesions and retroperitoneal bleeding. Molecular testing, including fluorescence in situ hybridization, of bone marrow and peripheral blood showed no evidence of PDGFR rearrangements. The patient was initially treated with high-dose steroid therapy and then with hydroxyurea, but proved unresponsive to both. Upon subsequent initiation of imatinib mesilate, the patient showed a dramatic improvement in eosinophil count and progressed rapidly through clinical recovery. Long-term follow-up confirmed the efficacy of treatment with low-dose imatinib and with no need of supplemental steroid treatment, notwithstanding the absence of PDGFR rearrangement.
Volume Number	4
PubMed Central reference number	PMC5067496
Issue Number	10
PubMed reference number	27803915
Journal	World Journal of Clinical Cases
e-ISSN	23078960
DOI	10.12998/wjcc.v4.i10.328
Language	English
Publisher	Baishideng Publishing Group Inc
Publisher Date	2016-10-01
Access Restriction	Open
Rights License	Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/ ©The Author(s) 2016. Published by Baishideng Publishing Group Inc. All rights reserved.
Subject Keyword	Idiopathic hypereosinophilic syndrome Eosinophilia Cerebral vasculitis PDGFR molecular rearrangement Imatinib mesilate
Content Type	Text
Resource Type	Article
Subject	Medicine