Division of Allergy and Immunology, Department of Medicine, Northwestern University Feinberg School of Medicine, 240 E. Huron Street, Room M306, Chicago, IL, 60611, USA.
Curr Hematol Malig Rep. 2018 Jun;13(3):191-201. doi: 10.1007/s11899-018-0448-8.
Hypereosinophilic syndrome (HES) is characterized by persistent hypereosinophilia associated with end-organ damage. As our understanding of the pathogenesis of various forms of HES broadens, so does our ability to tailor steroid-sparing therapies for each subtype. The purpose of this review is to summarize recent literature related to the etiology, diagnosis, and management of HES.
Mutations involved in subsets of HES can guide the choice of tyrosine kinase inhibitors beyond just imatinib. Several biologics that target interleukin-5 or its receptor have shown beneficial and selective eosinophil-reducing effects in clinical trials for asthma and other disorders including HES. Early clinical data with emerging therapies such as dexpramipexole and anti-Siglec-8 antibody show promise, but need to be confirmed in randomized trials. Several new biologics and tyrosine kinase inhibitors have been shown to lower eosinophil numbers, but more randomized trials are needed to confirm efficacy in HES.
嗜酸性粒细胞增多综合征(HES)的特征是持续的嗜酸性粒细胞增多,并伴有终末器官损伤。随着我们对各种形式 HES 发病机制的理解不断加深,我们也能够为每种亚型定制类固醇保留疗法。本文综述的目的是总结与 HES 的病因、诊断和治疗相关的最新文献。
HES 亚组中涉及的突变可以指导酪氨酸激酶抑制剂的选择,而不仅仅是伊马替尼。几种针对白细胞介素-5 或其受体的生物制剂在哮喘和其他疾病(包括 HES)的临床试验中显示出有益的、选择性的嗜酸性粒细胞减少作用。新兴疗法(如 dexpramipexole 和抗 Siglec-8 抗体)的早期临床数据显示出前景,但仍需在随机试验中得到证实。已经有几种新型生物制剂和酪氨酸激酶抑制剂被证明可以降低嗜酸性粒细胞数量,但仍需要更多的随机试验来证实其在 HES 中的疗效。