血小板源生长因子受体α相关高嗜酸性粒细胞综合征和淋巴瘤样丘疹病  

Platelet-derived growth factor receptor-α-associated hypereosinophilic syndrome and lymphomatoid papulosis

作　　者：McPherson T. Cowen E.W. McBurney E Klion A.D 冯义国 

机构地区：[1]National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD 20892, United States

出　　处：《世界核心医学期刊文摘（皮肤病学分册）》2006年第12期17-18,共2页Digest of the World Core Medical JOurnals:Dermatology

摘　　要：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.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 presen...

关 键 词：血小板源生长因子 嗜酸性粒细胞 淋巴瘤 受体Α 丘疹病 综合征 综合病症 

分 类 号：R758.6[医药卫生—皮肤病学与性病学]