Salm Cindy M, Clair Nicole E St, Lustig James V, Samyn Margaret M
Department of Medicine, Aurora Health Care, Milwaukee, WI 53213, USA.
J Allergy (Cairo). 2009;2009:804910. doi: 10.1155/2009/804910. Epub 2010 Jan 3.
Hypereosinophilic syndrome (HES) is a rare disorder typically seen in males, aged 20 to 50, with a predisposition for Caucasians. It is marked by overproduction of eosinophils (>1,500/μL) and multiorgan system damage due to eosinophilic infiltration and mediator release. There are multiple variants of HES. Cardiac complications are more common in myeloproliferative HES associated with the FIP1L1-PDGFRα mutation. Sequelae range from acute necrosis and thrombus formation to fibrosis of the endomyocardium. We describe a young boy who presented with chest pain and dyspnea. A diagnosis of HES was made after all other etiologies of eosinophilia were excluded. Although he was found to be negative for the FIP1L1-PDGFRα mutation, his cardiac complications included pericardial effusion and restrictive cardiomyopathy, without myocardial necrosis. Multi-organ involvement resulted in pericarditis, pleuritis, nephritis, and dermatitis. In this paper, we review his case and discuss the known subtypes of HES, the classic cardiac complications, and available treatment strategies.
高嗜酸性粒细胞综合征（HES）是一种罕见疾病，多见于20至50岁的男性，白种人更易患病。其特征为嗜酸性粒细胞过度生成（>1500/μL）以及因嗜酸性粒细胞浸润和介质释放导致的多器官系统损害。HES有多种变体。与FIP1L1 - PDGFRα突变相关的骨髓增殖性HES中，心脏并发症更为常见。后遗症范围从急性坏死和血栓形成到心内膜纤维化。我们描述了一名出现胸痛和呼吸困难的小男孩。在排除所有其他嗜酸性粒细胞增多的病因后，诊断为HES。尽管发现他FIP1L1 - PDGFRα突变呈阴性，但其心脏并发症包括心包积液和限制性心肌病，无心肌坏死。多器官受累导致了心包炎、胸膜炎、肾炎和皮炎。在本文中，我们回顾他的病例，并讨论HES的已知亚型、典型心脏并发症及可用的治疗策略。
