高嗜酸性粒细胞综合征的临床和免疫学生物标志物：诊断算法后的第二步。
Clinical and immunological biomarkers in hypereosinophilic syndrome: the second step after diagnostic algorithms.
作者信息
Longhino David, Baglivo Ilaria, Zavarella Maria Antonietta, Colantuono Stefania, Laface Chiara, Lucca Gabriele, Bruno Laura, Selvi Fabio Romano, Patella Vincenzo, Detoraki Aikaterini, Buonagura Rosa, Tatarelli Caterina, Moscatelli Barbara, D'Avelli Serena, Abruzzese Elisabetta, Greco Elisabetta, Gasbarrini Antonio, Pagano Livio, Criscuolo Marianna, Giammarco Sabrina, Caruso Cristiano
机构信息
UOSD Allergology and Clinical Immunology Unit, Fondazione Policlinico Universitario 'A. Gemelli' IRCCS, Università Cattolica del Sacro Cuore, Rome, Italy.
Centre for Digestive Diseases (CEMAD) and Gastroenterology Unit, Fondazione Policlinico Universitario A. Gemelli, IRCCS, Rome, Italy.
出版信息
Front Med (Lausanne). 2025 Jul 2;12:1600728. doi: 10.3389/fmed.2025.1600728. eCollection 2025.
BACKGROUND
Idiopathic hypereosinophilic syndrome currently represents a major unmet need for all medical specialties dealing with this disease. Markers capable of characterising the wide variability of its clinical presentation are currently lacking.
OBJECTIVE
This study aims to evaluate a panel of possible markers in idiopathic hypereosinophilic syndrome.
METHODS
In this pilot prospective single-centre cohort study, we analysed clinical (age, years of disease, steroid therapy) and laboratory (absolute eosinophil count, total IgE antibodies, IgE antibodies against Staphylococcus aureus enterotoxins, serum eosinophil cationic protein, serum immunoglobulin free light chains k and λ and their ratio) data obtained from 21 patients suffering from idiopathic hypereosinophilic syndrome from June 2023 to December 2024.
RESULTS
Mean absolute eosinophilic count was 3758.57 cells/μL. 17 patients were receiving treatment with > 7.5 mg of prednisone or equivalent at the time of the diagnosis. 13 patients had positive Staphylococcus aureus enterotoxins IgE, while the mean total serum IgE was 241.64 kU/L. We observed a high serum eosinophil cationic protein value as well as a high serum κ free light chain, while serum λ and κ/λ were normal. Patients with higher absolute eosinophilic count had higher eosinophil cationic protein levels ( < 0.05), such as higher steroid consumption ( < 0.05). In addition, we found a strong association between high κ free light chain levels and high previous steroid use and with Staphylococcus aureus enterotoxins IgE positivity.
CONCLUSION
Our results could increase the number of possible biomarkers for risk stratification in idiopathic hypereosinophilic syndrome.
背景
特发性嗜酸性粒细胞增多综合征目前是所有治疗该疾病的医学专科面临的一项重大未满足需求。目前缺乏能够表征其临床表现广泛变异性的标志物。
目的
本研究旨在评估一组特发性嗜酸性粒细胞增多综合征中可能的标志物。
方法
在这项前瞻性单中心队列试点研究中，我们分析了2023年6月至2024年12月期间从21例特发性嗜酸性粒细胞增多综合征患者获得的临床（年龄、病程、类固醇治疗）和实验室（绝对嗜酸性粒细胞计数、总IgE抗体、抗金黄色葡萄球菌肠毒素的IgE抗体、血清嗜酸性粒细胞阳离子蛋白、血清免疫球蛋白游离轻链κ和λ及其比值）数据。
结果
平均绝对嗜酸性粒细胞计数为3758.57个细胞/μL。17例患者在诊断时接受>7.5mg泼尼松或等效药物治疗。13例患者抗金黄色葡萄球菌肠毒素IgE呈阳性，而血清总IgE平均为241.64kU/L。我们观察到血清嗜酸性粒细胞阳离子蛋白值较高以及血清κ游离轻链较高，而血清λ和κ/λ正常。绝对嗜酸性粒细胞计数较高的患者嗜酸性粒细胞阳离子蛋白水平较高（<0.05），类固醇消耗量也较高（<0.05）。此外，我们发现高κ游离轻链水平与既往高类固醇使用量以及抗金黄色葡萄球菌肠毒素IgE阳性之间存在密切关联。
结论
我们的结果可能会增加特发性嗜酸性粒细胞增多综合征风险分层中可能的生物标志物数量。
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