Abstract
Idiopathic hypereosinophilic syndrome (HES) is a rare hematologic disorder characterized by persistent eosinophilia with organ involvement. Patients with HES have a poor prognosis, but the disease course can be heterogeneous. Treatment of HES has included corticosteroids, chemotherapeutic agents such as cyclophosphamide, vincristine, hydroxyrea, and most recently interferon-alpha (IFN-alpha) which has shown long-term beneficial effects. We herein report on a patient with HES who had disease resistant to steroids, and chemotherapy with 2-chlorodeoxyadenosine and cytarabine, but who had a significant response after only 8 days of treatment with imatinib mesylate 100mg daily. The possible mechanism of response is discussed. This observation may lead to a better understanding of the pathophysiology of HES, and may provide a new form of effective therapy for the disease.
MeSH terms
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Alkylating Agents / therapeutic use
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Antimetabolites / therapeutic use
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Benzamides
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Bone and Bones / blood supply
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Busulfan / therapeutic use
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Cladribine / therapeutic use
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Combined Modality Therapy
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Consciousness Disorders / etiology
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Cytarabine / therapeutic use
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Drug Resistance, Multiple
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Enzyme Inhibitors / pharmacology
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Enzyme Inhibitors / therapeutic use*
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Fatal Outcome
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Humans
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Hydroxyurea / therapeutic use
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Hypereosinophilic Syndrome / complications
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Hypereosinophilic Syndrome / drug therapy*
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Hypereosinophilic Syndrome / surgery
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Imatinib Mesylate
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Immunosuppressive Agents / therapeutic use
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Indomethacin / therapeutic use
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Infarction / etiology
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Male
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Middle Aged
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Mitral Valve Stenosis / etiology
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Pancytopenia / etiology
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Piperazines / therapeutic use*
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Protein-Tyrosine Kinases / antagonists & inhibitors*
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Pyrimidines / therapeutic use*
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Splenectomy
Substances
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Alkylating Agents
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Antimetabolites
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Benzamides
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Enzyme Inhibitors
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Immunosuppressive Agents
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Piperazines
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Pyrimidines
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Cytarabine
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Cladribine
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Imatinib Mesylate
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Protein-Tyrosine Kinases
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Busulfan
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Hydroxyurea
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Indomethacin