特发性嗜酸性粒细胞增多综合征15例临床分析

目的探讨特发性嗜酸性粒细胞增多综合征的临床特点、诊断及治疗,提高此类疾病的早期诊治水平。方法回顾性分析2010年9月至2018年5月在中日友好医院住院确诊为特发性嗜酸性粒细胞增多综合征患者的临床诊治资料,并结合相关文献复习加以总结归纳。结果共纳入15例特发性嗜酸性粒细胞增多综合征患者,女3例,男12例,平均年龄(53.7±21.3)岁。呼吸系统受累11例,平均病程7个月;胸部影像学多累及双肺,出现斑片影或磨玻璃影、胸腔积液、纵隔淋巴结增大为常见表现。血清总IgE多有明显升高。合并呼吸系统以外受累4例:消化系统损害1例,皮肤受累3例。患者外周血、骨髓及组织病理中嗜酸性粒细胞显著增高,此外可见患者痰、胸腔积液、支气管肺泡灌洗液的嗜酸性粒细胞增高,低氧血症多表现在有呼吸系统受累患者。经过糖皮质激素治疗后血嗜酸性粒细胞水平有减低趋势,低氧血症显著改善。结论特发性嗜酸性粒细胞增多综合征临床症状、体征、影像学缺乏特异性,漏诊、误诊率高;可有多系统受累,应尽早完善病理学检查明确诊断,防止出现多系统损害;应用糖皮质激素治疗有反应。

Clinical analysis of 15 cases of idiopathic hypereosinophilic syndrome

Objective To explore the clinical characteristics of idiopathic hypereosinophilic syndrome(IHES),and improve the early diagnosis and treatment of such diseases. Methods The clinical diagnosis and treatment data were retrospectively analyzed from the patients with confirmed IHES hospitalized in China-Japan Friendship Hospital between September 2010 to May 2018. Results Fifteen patients were included. There were 3 women and 12 men in the study, with an average age of 53.7±21.3 years. Eleven patients had respiratory problems, with an average course of 7 months. Most lesions occurred in both lungs. Patchy distribution, ground glass opacity, pleural effusion and mediastinal lymph node enlargement were common in the chest computed tomography. Serum total IgE was significantly increased. Four patients had other systems involved rather than respiratory system. One of them had digestive problems and another 3 had skin diseases. There was a significant increase in eosinophils in peripheral blood, bone marrow and histopathology, the same as eosinophils in sputum, pleural effusion, and bronchoalveolar lavage fluid. Hypoxemia was common in patients with respiratory problems. The blood eosinophil and total IgE were reduced after glucocorticoid treatment, and the hypoxemia was significantly improved. Conclusions The clinical symptoms, signs and image of chest computed tomography are not specific in IHES, so the rate of misdiagnosis and wrong diagnosis is high. This disease involves many organs or systems, so the pathological examination should be completed as soon as possible to make a clear diagnosis to prevent further damage.Glucocorticoid treatment is effective in this disease.