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 ...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.展开更多
嗜碱性粒细胞是白细胞的一种,起源于骨髓造血多能干细胞,在骨髓内分化成熟后进入血液。通过IgE高亲和力受体(high-affinity receptor for immunoglobulin E,FcεRI)激活的嗜碱性粒细胞在各种IgE介导的免疫反应中起关键作用,如哮喘、花...嗜碱性粒细胞是白细胞的一种,起源于骨髓造血多能干细胞,在骨髓内分化成熟后进入血液。通过IgE高亲和力受体(high-affinity receptor for immunoglobulin E,FcεRI)激活的嗜碱性粒细胞在各种IgE介导的免疫反应中起关键作用,如哮喘、花粉症、食物过敏等。近年来,越来越多的研究表明嗜碱性粒细胞在慢性自发性荨麻疹的发病机制中同样起着重要作用。本文就嗜碱性粒细胞在慢性自发性荨麻疹发病机制中的作用进行综述。展开更多
文摘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.
文摘嗜碱性粒细胞是白细胞的一种,起源于骨髓造血多能干细胞,在骨髓内分化成熟后进入血液。通过IgE高亲和力受体(high-affinity receptor for immunoglobulin E,FcεRI)激活的嗜碱性粒细胞在各种IgE介导的免疫反应中起关键作用,如哮喘、花粉症、食物过敏等。近年来,越来越多的研究表明嗜碱性粒细胞在慢性自发性荨麻疹的发病机制中同样起着重要作用。本文就嗜碱性粒细胞在慢性自发性荨麻疹发病机制中的作用进行综述。
