Abstract
Fip1-like 1/platelet-derived growth factor receptor-alpha (FIP1L1/PDGFRA)-positive hypereosinophilic syndrome is a rare disorder with a poor prognosis if untreated and for which treatment with imatinib mesilate is highly effective. A 33-year-old man presented with recurrent papular skin lesions and marked peripheral eosinophilia. Skin biopsy revealed proliferation of CD30(+) T cells consistent with lymphomatoid papulosis (LyP), whereas molecular analysis of peripheral blood mononuclear cells demonstrated the presence of the FIP1L1/PDGFRA fusion gene. As the presence of this gene has important prognostic and therapeutic implications, this report underscores the importance of molecular testing in the evaluation of patients with LyP and peripheral eosinophilia.
MeSH terms
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Adult
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Benzamides
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Humans
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Hypereosinophilic Syndrome / diagnosis
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Hypereosinophilic Syndrome / drug therapy
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Hypereosinophilic Syndrome / genetics*
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Imatinib Mesylate
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Lymphomatoid Papulosis / diagnosis
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Lymphomatoid Papulosis / drug therapy
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Lymphomatoid Papulosis / genetics*
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Male
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Oncogene Proteins, Fusion / genetics*
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Piperazines / therapeutic use
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Protein Kinase Inhibitors / therapeutic use
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Pyrimidines / therapeutic use
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Receptor, Platelet-Derived Growth Factor alpha / genetics*
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mRNA Cleavage and Polyadenylation Factors / genetics*
Substances
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Benzamides
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Oncogene Proteins, Fusion
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Piperazines
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Protein Kinase Inhibitors
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Pyrimidines
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mRNA Cleavage and Polyadenylation Factors
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Imatinib Mesylate
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FIP1L1-PDGFRA fusion protein, human
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Receptor, Platelet-Derived Growth Factor alpha