De Vriese A S, Kips J C, Vogelaers D P, Vandewoude K H, Cuvelier C A, Colardyn F A
Department of Intensive Care Medicine, University Hospital, Ghent, Belgium.
J Intern Med. 1997 Feb;241(2):165-70. doi: 10.1046/j.1365-2796.1997.73100000.x.
The idiopathic hypereosinophilic syndrome is empirically defined as the presence of prolonged eosinophilia without identifiable underlying cause, and with evidence of end-organ dysfunction. Virtually any organ system may be involved, most frequently the heart, the central and peripheral nervous system, the lungs and the skin. We report two cases where the diagnosis of hypereosinophilic syndrome was proposed although the classic criteria were not met. In the first case total peripheral eosinophil counts were relatively low, but pathological evidence clearly showed infiltration of eosinophils in the damaged tissues. An hypothesis to explain this discrepancy is formulated. The second case did not fulfil the first feature either, although the clinical presentation and disease course corresponded well with other cases reported in the literature. The delay in diagnosis was caused by early institution of corticosteroids, clearing all evidence of eosinophil involvement in the observed tissue damage.
特发性嗜酸性粒细胞增多综合征根据经验定义为存在持续性嗜酸性粒细胞增多且无明确的潜在病因，并伴有终末器官功能障碍的证据。几乎任何器官系统都可能受累，最常见的是心脏、中枢和外周神经系统、肺和皮肤。我们报告了两例病例，尽管未满足经典标准，但仍提出了嗜酸性粒细胞增多综合征的诊断。在第一例病例中，外周血嗜酸性粒细胞总数相对较低，但病理证据清楚地显示受损组织中有嗜酸性粒细胞浸润。本文提出了一个解释这种差异的假说。第二例病例也不满足第一个特征，尽管其临床表现和病程与文献中报道的其他病例非常吻合。诊断延迟是由于早期使用了皮质类固醇，清除了观察到的组织损伤中嗜酸性粒细胞受累的所有证据。
