目的探讨原发嗜酸细胞增多症的临床特点。方法回顾性分析55例原发嗜酸细胞增多症患者的临床资料。结果55例均符合原发嗜酸细胞增多症的诊断标准,男性为女性的2倍。胃肠道异常为最常见的临床表现(50.8%),其次为肺部病变(37.7%)、心脏病变(26.2%)和神经系统病变(21.3%);其他临床表现包括皮肤改变(19.7%)、浅表淋巴结肿大(18%)、血栓形成(9.8%)和哮喘(3.3%)。肠黏膜和浅表淋巴结活检均表现为大量嗜酸细胞浸润,经糖皮质激素治疗后临床表现均明显好转。结论男性较女性更易患原发嗜酸细胞增多症,最常见的受累器官为胃肠道、肺、心脏和神经系统,糖皮质激素治疗有效。 Objective To investigate the clinical features of primary eosinophilia. Methods The clinical data of 55 patients with primary eosinophilia were retrospectively analyzed. Results All 55 patients met the diagnostic criteria of primary eosinophilia, which was twice as high as that of men. Gastrointestinal abnormalities were the most common clinical manifestation (50.8%), followed by lung disease (37.7%), heart disease (26.2%) and nervous system diseases (21.3%). Other clinical manifestations included skin changes (19.7% , Superficial lymph nodes (18%), thrombosis (9.8%) and asthma (3.3%). Intestinal mucosa and superficial lymph node biopsy showed a large number of eosinophilic infiltration, the clinical manifestations after glucocorticoid treatment were significantly improved. Conclusions Men are more susceptible to primary eosinophilia than women. The most common organ involvement is gastrointestinal tract, lung, heart and nervous system, and glucocorticoid therapy is effective.