Mercadanti M, Cavalieri F, Ferraccioli G F, Ambanelli U
Minerva Med. 1985 Oct 13;76(39):1781-90.
Idiopathic hypereosinophilic syndrome is a term labelling clinical illnesses characterized by blood hypereosinophilia and widespread infiltration of organs and tissues by mature eosinophils. Although any tissue can be affected the involvement of the nervous system and of the myocardium usually indicates worse prognosis. Histopathological studies carried out on myocardial tissues of hypereosinophilic patients, suggest that eosinophils are the main inducers of tissue damages. These cells seem to affect first the coronary endothelial cells, so leading to thrombi formation and then fibrosis. Eventually restrictive cardiomyopathy may occur. The pathogenetic events are not fully understood. However several studies have been focused on two eosinophil proteins: the major basic protein (MBP) as possible mediator of tissue damages and the cationic eosinophil protein (ECP) involved in thrombotic events. In the bone marrow a marked hyperplasia of the eosinophil series is usual, while in the blood only mature eosinophils circulate. Non specific abnormalities either of the cellular or humoral immunity have been described. Previous reports of a definitely incurable disease have been improved by modern therapeutic programs.
特发性嗜酸性粒细胞增多综合征是一个用于描述临床疾病的术语，其特征为血液嗜酸性粒细胞增多以及成熟嗜酸性粒细胞广泛浸润器官和组织。尽管任何组织都可能受累，但神经系统和心肌受累通常提示预后较差。对嗜酸性粒细胞增多患者心肌组织进行的组织病理学研究表明，嗜酸性粒细胞是组织损伤的主要诱导因素。这些细胞似乎首先影响冠状动脉内皮细胞，从而导致血栓形成，进而纤维化。最终可能发生限制性心肌病。发病机制尚未完全明了。然而，多项研究聚焦于两种嗜酸性粒细胞蛋白：作为可能的组织损伤介质的主要碱性蛋白（MBP）以及参与血栓形成事件的阳离子嗜酸性粒细胞蛋白（ECP）。在骨髓中，嗜酸性粒细胞系列通常有明显增生，而血液中仅循环成熟嗜酸性粒细胞。已描述了细胞免疫或体液免疫的非特异性异常。现代治疗方案改善了以往关于该病绝对无法治愈的报道。
