嗜酸性粒细胞增多症患者心脏受累的临床和病理表现

The clinico-pathological manifestation of cardiac involvement in eosinophilic diseases

目的 探讨嗜酸性粒细胞增多症心脏受累的临床和病理特点.方法 分析7例嗜酸性粒细胞增多症累及心脏且行心内膜活检或尸检患者的临床和心肌病理资料.结果 7例患者中5例为男性,平均年龄51岁;特发性嗜酸性粒细胞增多综合征4例,Churg-Strauss综合征3例.7例患者外周血嗜酸性粒细胞均有明显升高.心脏受累的表现有心绞痛、心肌梗死、心肌病变合并心功能不全、瓣膜病变合并严重心功能不全、房室传导阻滞和猝死.病理显示有嗜酸性粒细胞浸润、肉芽肿形成、坏死性血管炎、心肌细胞坏死、间质纤维化和心内膜纤维化.经糖皮质激素和免疫抑制剂治疗后大部分患者外周血嗜酸性粒细胞降低或正常,心脏病变稳定甚至好转.结论 嗜酸性粒细胞增多症伴发心脏受累的临床表现多样而且预后差;早期识别并积极治疗有助于改善患者的预后.

Objective To investigate the clinical and pathological features of eosinophilic diseases with cardiac involvement Methods We analyzed the clinical and cardiac pathological data of 7 patients with eosinophilic diseases with cardiac involvement under endomyocardial biopsy or autopsy.Results Seven patients （5 male, average age 51 years） were enrolled.Four patients were diagnosed as idiopathic hypereosinophilic syndrome and three were Churg-Strauss syndrome.Peripheral blood eosinophila count increased significantly in all patients.Cardiac involvement included angina pectoris, myocardial infarction, heart failure, presyncope and sudden death.Electrocardiogram showed cardiac ischemia, bundle branch block and third degree atrioventricular block.Echocardiography suggested ventricular and atrial enlargement, decreased ventricular systolic function, pulmonary hypertension, valvular prolapse and insufficiency and endocarditis.Pathology displayed infiltration of eosinophils, formation of granulomata, necrotizing vasculitis, myocardial necrosis and endomyocardial fibrosis in heart.Coronary artery could be also affected and led to myocardial infarction.Conclusions Cardiovascular complications of eocinophilic diseases are a major source of morbidity and mortality in these disorders.The manifestations are multiple and early recognition and treatment with steroid and immunosuppressant can improve prognosis.