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高嗜酸性粒细胞综合征的临床及实验室特征研究

Clinical and laboratory characteristics of hypereosinophilic syndrome
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摘要 目的探讨高嗜酸性粒细胞综合征(HES)各亚型的临床及实验室特点,尤其是骨髓及外周血涂片中嗜酸性粒细胞的形态学特征。方法回顾性分析该院2017-2020年63例HES患者的临床和实验室资料,复检外周血和骨髓涂片中嗜酸性粒细胞的形态学特征。结果63例HES患者中有反应性HES患者54例,特发性HES患者9例。反应性HES患者的病因众多,主要为肿瘤(嗜酸性粒细胞为非克隆性)、血管炎、感染性疾病及药物等。两组HES患者的临床表现均以皮疹皮炎最为常见,脾肿大及骨髓纤维化发生率接近(P>0.05)。特发性HES患者的外周血嗜酸性粒细胞比例高于反应性HES患者(P<0.05)。33%的特发性HES患者外周血可出现幼稚嗜酸性粒细胞,而反应性HES患者未见(P<0.05)。骨髓涂片中特发性HES患者的嗜酸性粒细胞更易出现形态异常,其中特发性HES患者嗜酸性粒细胞胞质颗粒稀疏、胞质嗜碱性颗粒及核分叶过多这3种异常形态的发生率高于反应性HES患者(P<0.05)。结论特发性和反应性HES的临床及实验室特征相互重叠,但通过骨髓涂片中嗜酸性粒细胞的异常形态学特征,能够为两种亚型鉴别诊断提供一定帮助。 Objective To investigate the clinical and laboratory characteristics of various subtypes of hypereosinophilic syndrome(HES),especially the abnormal morphological characteristics of eosinophils in bone marrow and peripheral blood smears.Methods The clinical and laboratory data of 63 patients with HES in our hospital from 2017 to 2020 were retrospectively analyzed,and the morphological characteristics of eosinophils in peripheral blood and bone marrow smears were reviewed.Results Among the 63 HES patients,54 were reactive HES patients and 9 were idiopathic HES patients.There were many etiologies in patients with reactive HES,mainly were tumors(eosinophils were non-clonal),vasculitis,infectious diseases,and drugs.The most common clinical manifestations of HES patients were rash dermatitis,with similar incidences of splenomegaly and myelofibrosis in the two groups(P>0.05).The proportion of peripheral blood eosinophils in patients with idiopathic HES was higher than that in patients with reactive HES(P<0.05).33%of idiopathic HES patients had naive eosinophils in peripheral blood,but none of reactive HES patients(P<0.05).The eosinophils in idiopathic HES patients were more likely to have abnormal morphology in bone marrow smears.The incidence of esosinophils with sparse granules,cytoplasmic basophilic granules and nuclear hyperlobation was higher in patients with idiopathic HES than in patients with reactive HES(P<0.05).Conclusion The clinical and laboratory features of idiopathic and reactive HES overlap each other,but the abnormal morphological features of eosinophils in bone marrow smears can provide some help in the differential diagnosis of the two subtypes.
作者 张岚 朱建锋 陈朴 郭玮 王蓓丽 ZHANG Lan;ZHU Jianfeng;CHEN Pu;GUO Wei;WANG Beili(Department of Clinical Laboratory,Zhongshan Hospital Affiliated to Fudan University,Shanghai 200032,China)
出处 《国际检验医学杂志》 CAS 2022年第3期257-261,共5页 International Journal of Laboratory Medicine
基金 国家自然科学基金面上项目(81772263、81972000) 国家自然科学基金青年项目(81902139) 2019厦门市医疗卫生重点项目(YDZX20193502000002) 复旦大学附属中山医院院内临床研究项目(2018ZSLC05)。
关键词 高嗜酸性粒细胞综合征 特发性高嗜酸性粒细胞综合征 反应性高嗜酸性粒细胞综合征 异常形态 hypereosinophilic syndrome idiopathic hypereosinophilic syndrome reactive hypereosinophilic syndrome abnormal morphology
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