Fauci A S, Harley J B, Roberts W C, Ferrans V J, Gralnick H R, Bjornson B H
Ann Intern Med. 1982 Jul;97(1):78-92. doi: 10.7326/0003-4819-97-1-78.
The idiopathic hypereosinophilic syndrome (HES) represents a heterogeneous group of disorders with the common features of prolonged eosinophilia of an undetectable cause and organ system dysfunction. Fifty patients with the idiopathic HES were studied over 11 years of the National Institutes of Health. Multiple organ systems were involved; bone marrow hypereosinophilia was common to all patients, but the most severe clinicopathologic involvement was of the heart and nervous system. Postmortem gross pathologic examination of the hearts of patients with idiopathic and nonidiopathic HES suggested that the common mechanism of cardiac disease is the eosinophilia. Endomyocardial biopsy findings showed that the endothelial cells in the endocardium and of the microvasculature were the primary targets of the tissue damage. This damage initiates thrombosis; endocardial fibrosis and restrictive endomyocardopathy may follow. In-vitro culture of circulating eosinophil colony-forming units showed some normal studies, some studies showing increased progenitor cells committed to eosinophil development, and others showing an excess production of eosinophil colony-stimulating factor. Chemotherapy to lower the eosinophil counts has resulted in marked improvement of HES prognosis, as have agressive medical and surgical approaches to cardiovascular complications.
特发性高嗜酸性粒细胞综合征（HES）是一组异质性疾病，其共同特征为病因不明的持续性嗜酸性粒细胞增多及器官系统功能障碍。美国国立卫生研究院在11年期间对50例特发性HES患者进行了研究。多个器官系统受累；所有患者均有骨髓嗜酸性粒细胞增多，但最严重的临床病理受累部位是心脏和神经系统。对特发性和非特发性HES患者心脏的尸检大体病理检查表明，心脏疾病的共同机制是嗜酸性粒细胞增多。心内膜活检结果显示，心内膜和微血管的内皮细胞是组织损伤的主要靶点。这种损伤引发血栓形成；随后可能出现心内膜纤维化和限制性心内膜心肌病。循环嗜酸性粒细胞集落形成单位的体外培养显示，一些研究结果正常，一些研究显示致力于嗜酸性粒细胞发育的祖细胞增多，还有一些研究显示嗜酸性粒细胞集落刺激因子产生过多。降低嗜酸性粒细胞计数的化疗已显著改善了HES的预后，积极的药物和手术治疗心血管并发症也有同样效果。
