Idiopathic hypereosinophilic syndrome presenting with severe vasculitis successfully treated with imatinib.
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 withFIP1L1-PDGFRAgene 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 fluorescencein situhybridization, of bone marrow and peripheral blood showed no evidence ofPDGFRrearrangements. 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 ofPDGFRrearrangement.
Paolo Fraticelli, Alain Kafyeke, Massimo Mattioli, Giuseppe Pio Martino, Marta Murri, Armando Gabrielli, Clinica Medica, Department of Internal Medicine, Università Politecnica delle Marche, Ospedali Riuniti, 60020 Ancona, Italy.
Paolo
P
Fraticelli
Paolo Fraticelli, Alain Kafyeke, Massimo Mattioli, Giuseppe Pio Martino, Marta Murri, Armando Gabrielli, Clinica Medica, Department of Internal Medicine, Università Politecnica delle Marche, Ospedali Riuniti, 60020 Ancona, Italy.
Alain
A
Kafyeke
Paolo Fraticelli, Alain Kafyeke, Massimo Mattioli, Giuseppe Pio Martino, Marta Murri, Armando Gabrielli, Clinica Medica, Department of Internal Medicine, Università Politecnica delle Marche, Ospedali Riuniti, 60020 Ancona, Italy.
Massimo
M
Mattioli
Paolo Fraticelli, Alain Kafyeke, Massimo Mattioli, Giuseppe Pio Martino, Marta Murri, Armando Gabrielli, Clinica Medica, Department of Internal Medicine, Università Politecnica delle Marche, Ospedali Riuniti, 60020 Ancona, Italy.
Giuseppe Pio
GP
Martino
Paolo Fraticelli, Alain Kafyeke, Massimo Mattioli, Giuseppe Pio Martino, Marta Murri, Armando Gabrielli, Clinica Medica, Department of Internal Medicine, Università Politecnica delle Marche, Ospedali Riuniti, 60020 Ancona, Italy.
Marta
M
Murri
Paolo Fraticelli, Alain Kafyeke, Massimo Mattioli, Giuseppe Pio Martino, Marta Murri, Armando Gabrielli, Clinica Medica, Department of Internal Medicine, Università Politecnica delle Marche, Ospedali Riuniti, 60020 Ancona, Italy.
Armando
A
Gabrielli
Cerebral vasculitis; Eosinophilia; Idiopathic hypereosinophilic syndrome; Imatinib mesilate; PDGFR molecular rearrangement