Abstract
We report an unusual case of a symptomatic patient who initially had high hemoglobin and low serum erythropoietin levels, fitting a clinical diagnosis of polycythemia vera. However, after treatment with hydroxyurea and serial phlebotomies had been started, the patient developed hypereosinophilia, fitting the category of a myeloproliferative neoplasm with eosinophilia associated with the FIP1L1-PDGFRA gene fusion, as confirmed by molecular analysis. We discuss the clinical presentation, evolution, response to treatment, and pathogenetic implications of this case.
Copyright © 2012 Elsevier Inc. All rights reserved.
MeSH terms
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Antineoplastic Agents / therapeutic use
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Benzamides
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Biopsy
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Eosinophilia / complications
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Eosinophilia / diagnosis*
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Eosinophilia / genetics*
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Genetic Predisposition to Disease
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Humans
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Imatinib Mesylate
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Male
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Middle Aged
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Models, Genetic
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Mutation
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Myeloproliferative Disorders / complications
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Myeloproliferative Disorders / diagnosis
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Myeloproliferative Disorders / genetics*
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Piperazines / therapeutic use
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Polycythemia Vera / complications
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Polycythemia Vera / diagnosis*
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Polycythemia Vera / genetics*
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Pyrimidines / therapeutic use
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Receptor, Platelet-Derived Growth Factor alpha / genetics*
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Remission Induction
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mRNA Cleavage and Polyadenylation Factors / genetics*
Substances
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Antineoplastic Agents
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Benzamides
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FIP1L1 protein, human
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Piperazines
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Pyrimidines
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mRNA Cleavage and Polyadenylation Factors
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Imatinib Mesylate
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Receptor, Platelet-Derived Growth Factor alpha
Supplementary concepts
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Myeloproliferative Disorder, Chronic, with Eosinophilia