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2016 ; 4
(10
): 328-332
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Idiopathic hypereosinophilic syndrome presenting with severe vasculitis
successfully treated with imatinib
#MMPMID27803915
Fraticelli P
; Kafyeke A
; Mattioli M
; Martino GP
; Murri M
; Gabrielli A
World J Clin Cases
2016[Oct]; 4
(10
): 328-332
PMID27803915
show ga
Idiopathic hypereosinophilic syndrome (HES) is a rare disorder characterized by
peripheral eosinophilia exceeding 1500/mm(3), 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.