Division of Cardiology, Mayo Clinic, Rochester, Minnesota, USA.
Heart. 2016 Jan;102(2):100-6. doi: 10.1136/heartjnl-2015-307959. Epub 2015 Nov 13.
Hypereosinophilic syndrome (HES) is a heterogeneous group of conditions that is defined at its core by hypereosinophilia (HE) (blood eosinophil count of >1.5×10(9)/L) and organ damage directly attributable to the HE. Cardiac dysfunction occurs frequently in all forms of HES and is a major cause of morbidity and mortality. Once a significantly elevated eosinophil count is identified, it must be confirmed on repeat testing and the aetiology for the HE must be rigorously sought out with a focus on identifying whether organ dysfunction is occurring. Echocardiography is routinely performed to assess for cardiac involvement, looking for evidence of left ventricular and/or right ventricular apical obliteration or thrombi or a restrictive cardiomyopathy. Cardiac magnetic resonance imaging and CT are often useful adjuncts to establish the diagnosis but endomyocardial biopsy remains the gold standard. To decrease the degree of eosinophilia, treatment can include corticosteroids and/or imatinib based on the aetiology. Anticoagulation, standard heart failure therapy for a restrictive cardiomyopathy and finally cardiac transplantation may be indicated in the treatment algorithm.
嗜酸性粒细胞增多综合征（HES）是一组异质性疾病，其核心特征是嗜酸性粒细胞增多症（HE）（外周血嗜酸性粒细胞计数＞1.5×10(9)/L）和直接归因于 HE 的器官损伤。心脏功能障碍在所有类型的 HES 中都很常见，是发病率和死亡率的主要原因。一旦发现显著升高的嗜酸性粒细胞计数，必须在重复检测中确认，并严格寻找 HE 的病因，重点是确定是否发生器官功能障碍。超声心动图通常用于评估心脏受累情况，寻找左心室和/或右心室心尖闭塞或血栓形成或限制型心肌病的证据。心脏磁共振成像和 CT 通常有助于确诊，但心内膜心肌活检仍然是金标准。为了降低嗜酸性粒细胞增多的程度，可根据病因选择使用皮质类固醇和/或伊马替尼进行治疗。抗凝、限制型心肌病的标准心力衰竭治疗以及心脏移植可能是治疗方案中的指征。
