AI Article Synopsis
- FIP1L1/PDGFRA-positive hypereosinophilic syndrome is a rare but serious disorder that can worsen without treatment, and imatinib mesilate is an effective therapy.
- A case study of a 33-year-old man showed skin lesions and high eosinophil levels, with a biopsy revealing T cell proliferation linked to lymphomatoid papulosis.
- Molecular analysis revealed a fusion gene, highlighting the critical role of genetic testing in diagnosing and managing conditions that present with lymphomatoid papulosis and eosinophilia.
Article 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.
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| http://dx.doi.org/10.1111/j.1365-2133.2006.07416.x | DOI Listing |
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