高嗜酸性综合症（HES）的定义是指外周嗜酸性粒细胞计数>1500个/μL（在≥2周的间隔内进行两次评估），并伴有嗜酸性粒细胞引起的器官损伤。根据病因，原发性（克隆性或肿瘤性）HES和继发性（反应性）HES与特发性HES有所区别。多血管炎性肉芽肿性嗜酸性粒细胞增多症（EGPA）被归类为HES的继发性形式，其特征为高嗜酸性粒细胞计数，小到中等血管的血管炎，可能与抗中性粒细胞胞浆抗体（ANCA）相关。HES的治疗取决于病因。克隆性HES根据相应的基因异常进行治疗，例如使用酪氨酸激酶抑制剂或化疗和异基因造血干细胞移植。应根据潜在的原因（例如寄生虫感染）治疗继发性形式。EGPA的治疗取决于疾病阶段和活动程度的免疫抑制剂。通常使用传统药物，例如糖皮质激素（GC）、环磷酰胺（CYC）和甲氨蝶呤（MTX）或生物制剂，例如单克隆抗-IL5抗体美妥珠单抗。美妥珠单抗也是治疗特发性HES的良好选择。© 2023年。作者（根据Springer Medizin Verlag GmbH和Springer Nature的独家许可）

Hypereosinophilic syndrome (HES) is defined as a peripheral eosinophil count of > 1500/μl (assessed twice at an interval of ≥ 2 weeks) and an eosinophil-induced organ damage. Idiopathic HES is differentiated from primary (clonal or neoplastic) HES and secondary (reactive) HES, depending on the etiology. Eosinophilic granulomatosis with polyangiitis (EGPA) is categorized as a secondary form of HES and is characterized by hypereosinophilia and vasculitis of small to medium-sized vessels and can be associated with an antineutrophil cytoplasmic antibody (ANCA). The treatment of HES is dependent on the etiology. Clonal HES is treated according to the respective genetic aberration, e.g. with tyrosine kinase inhibitors or chemotherapy and allogenic stem cell transplantation. Secondary forms should be treated according to the underlying cause (e.g. parasitic infection). The treatment of EGPA is carried out with immunosuppressants depending on the disease stage and disease activity. Conventional drugs, such as glucocorticoids (GC), cyclophosphamide (CYC) and methotrexate (MTX) or biologics, such as the monoclonal anti-IL5 antibody mepolizumab are commonly used. Mepolizumab is also a good option for the treatment of idiopathic HES.© 2023. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.