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嗜酸性粒细胞性心内膜疾病的非典型表现。

Atypical presentation of eosinophilic endomyocardial disease.

作者信息

Blauwet Lori A, Breen Jerome F, Edwards William D, Klarich Kyle W

机构信息

Division of Cardiovascular Diseases , Mayo Clinic College of Medicine, Rochester, MN 55905, USA.

出版信息

Mayo Clin Proc. 2005 Aug;80(8):1078-84. doi: 10.4065/80.8.1078.

Abstract

Idiopathic hypereosinophllic syndrome is classically defined as prolonged, unexplained peripheral eosinophilia in a patient presenting with evidence of end-organ damage. The heart is frequently Involved, resulting In eosinophilic endomyocardial disease and eventually restrictive cardlomyopathy. The mortality rate is high because of progressive heart failure or ventricular arrhythmias. We describe a patient who presented with a left ventricular apical thrombus without notable peripheral eosinophilia. Findings from clinical evaluation and extensive diagnostic testing, including right ventricular biopsy, were Inconclusive. Resection of the thrombus and subjacent endomyocardium revealed eosinophilic Infiltration of the endomyocardium, which led to the diagnosis of eosinophilic endomyocardial disease. Clinicians should be aware of the variable presentation of patients with eosinophil-associated endomyocardial disease so that affected patients may benefit from early diagnosis and treatment.

摘要

特发性嗜酸性粒细胞增多综合征的经典定义是,患者出现终末器官损害证据,同时伴有持续存在且无法解释的外周嗜酸性粒细胞增多。心脏常受累,导致嗜酸性粒细胞性心内膜疾病,最终发展为限制性心肌病。由于进行性心力衰竭或室性心律失常,死亡率很高。我们描述了一名患者,其左心室心尖部有血栓形成,但外周嗜酸性粒细胞无明显增多。临床评估及包括右心室活检在内的广泛诊断检查结果均不明确。切除血栓及下方的心内膜显示心内膜有嗜酸性粒细胞浸润,从而确诊为嗜酸性粒细胞性心内膜疾病。临床医生应了解嗜酸性粒细胞相关性心内膜疾病患者的表现多样,以便使受影响的患者能够从早期诊断和治疗中获益。

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