Taurisano Giusi, Ruffi Maria Clara, Canalis Silvia, Costanzo Giulia Anna Maria Luigia
Department of Medical Sciences and Public Health, University of Cagliari, Cagliari, Italy.
Curr Opin Allergy Clin Immunol. 2025 Aug 1;25(4):258-268. doi: 10.1097/ACI.0000000000001078. Epub 2025 May 21.
This review addresses the clinical and biological complexities of hypereosinophilia (HE) and hypereosinophilic syndrome (HES), highlighting the need for improved diagnostic frameworks and therapeutic strategies. Due to the increasing recognition of HE and its potential for severe multiorgan involvement, a structured, multidisciplinary approach to diagnosis and management is essential for optimizing patient outcomes.
Recent literature categorizes HE into hereditary, reactive, and neoplastic forms, with significant advancements in defining associated conditions and their pathophysiological mechanisms. Clinical manifestations range from asymptomatic eosinophilia to life-threatening complications involving the skin, lungs, gastrointestinal tract, heart, and nervous system. Corticosteroids remain the first-line treatment across most subtypes. Imatinib has shown high efficacy, particularly in patients with FIP1L1::PDGFRA fusion. However, therapeutic resistance and relapse still occur. Biologic therapies targeting interleukin (IL)-5 or its receptor, such as mepolizumab and benralizumab, have demonstrated promise in reducing eosinophils counts and preventing flare-ups. Additional agents under investigation include dupilumab and lirentelimab.
The findings highlight the importance of accurate classification and tailored management of HE and HES, which are crucial for preventing organ damage and improving quality of life. Ongoing clinical trials and research will expand therapeutic options, clarify underlying mechanisms, and address current unmet needs.
本综述探讨了嗜酸性粒细胞增多症(HE)和嗜酸性粒细胞增多综合征(HES)的临床及生物学复杂性,强调了改进诊断框架和治疗策略的必要性。由于对HE及其严重多器官受累可能性的认识不断提高,采用结构化、多学科的方法进行诊断和管理对于优化患者预后至关重要。
近期文献将HE分为遗传性、反应性和肿瘤性三种类型,在确定相关疾病及其病理生理机制方面取得了重大进展。临床表现从无症状嗜酸性粒细胞增多到涉及皮肤、肺、胃肠道、心脏和神经系统的危及生命的并发症不等。皮质类固醇仍然是大多数亚型的一线治疗药物。伊马替尼已显示出高疗效,特别是在伴有FIP1L1::PDGFRA融合的患者中。然而,治疗抵抗和复发仍然会发生。靶向白细胞介素(IL)-5或其受体的生物疗法,如美泊利单抗和贝那利珠单抗,在降低嗜酸性粒细胞计数和预防病情复发方面已显示出前景。正在研究的其他药物包括度普利尤单抗和利仑替利单抗。
这些发现凸显了对HE和HES进行准确分类和个体化管理的重要性,这对于预防器官损伤和改善生活质量至关重要。正在进行的临床试验和研究将扩大治疗选择、阐明潜在机制并满足当前未满足的需求。