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Clinical Features of Hypereosinophilic Syndrome: FIP1L1-PDGFRA Fusion Gene—Positive Disease is a Distinct Clinical Entity with Myeloproliferative Features and a Poor Response to Corticosteroid

We conducted a retrospective analysis of the clinical features of 20 patients with severe eosinophilia at our institution, including 10 cases of hypereosinophilic syndrome (HES) (5 definite and 5 probable cases) and 10 cases of other eosinophilic disorders. Of the 20 patients, 14 initially received prednisolone treatment, which resulted in rapid improvement and normalization of eosinophilia within 8 weeks; however, 2 patients with splenomegaly showed poor control of eosinophilia in response to corticosteroid treatment. In addition, the FIP1L1-PDGFRA fusion gene was detected only in these 2 cases. One of the FIP1L1-PDGFRA—positive HES cases featured bone marrow fibrosis. Treatment of this patient with imatinib mesylate resulted in a dramatic improvement of eosinophilia, organomegaly, and the bone marrow fibrosis.Taken together, our data and previous reports suggest that FIP1L1-PDGFRA—positive HES is a distinct clinical entity with myeloproliferative features and showing a poor response to corticosteroid treatment.