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Platelet‐derived growth factor receptor‐ α ‐associated hypereosinophilic syndrome and lymphomatoid papulosis
Author(s) -
McPherson T.,
Cowen E.W.,
McBurney E.,
Klion A.D.
Publication year - 2006
Publication title -
british journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.304
H-Index - 179
eISSN - 1365-2133
pISSN - 0007-0963
DOI - 10.1111/j.1365-2133.2006.07416.x
Subject(s) - lymphomatoid papulosis , hypereosinophilic syndrome , medicine , pdgfra , eosinophilia , skin biopsy , pathology , imatinib mesylate , biopsy , immunology , lymphoma , imatinib , cd30 , stromal cell , gist , myeloid leukemia
Summary Fip1‐like 1/platelet‐derived growth factor receptor‐ α (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.