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伴有多器官受累的特发性高嗜酸性粒细胞综合征

Idiopathic Hypereosinophilic Syndrome with Multiple Organ Involvement.

作者信息

Abo Shdid Reham, Azrieh Bahjat, Alebbi Seham, Mansour Shafik, Naeem Muhammad

机构信息

Department of Medical Education, Hamad Medical Corporation, Doha, Qatar.

Hamad General Hospital, Hamad Medical Corporation, Doha, Qatar.

出版信息

Case Rep Oncol. 2021 Mar 2;14(1):249-255. doi: 10.1159/000511396. eCollection 2021 Jan-Apr.

DOI:10.1159/000511396
PMID:33776712
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7983584/
Abstract

Idiopathic hypereosinophilic syndrome is a rare disease which is diagnosed after excluding other conditions. The syndrome is characterized by multiple organ involvement including the heart, nervous system, lungs, and gastrointestinal tract. The disease is suspected if there is peripheral blood eosinophilia and no clear etiology. The main treatment is corticosteroids. Patients who do not respond to corticosteroids can be treated with imatinib, immunomodulatory agents, myelosuppressive therapy, or mepolizumab. Alemtuzumab can be considered in severe cases that are unresponsive to other therapies. In this paper, we describe a case of idiopathic hypereosinophilic syndrome with mainly cardiac system involvement and left ventricular thrombus formation which was complicated by cerebral thromboemboli while on warfarin with international normalized ratio in the therapeutic range. Our patient responded well to steroids appreciated by improvement in clinical symptoms and decrease in eosinophil count.

摘要

特发性高嗜酸性粒细胞综合征是一种罕见疾病,在排除其他病症后得以诊断。该综合征的特征是多器官受累,包括心脏、神经系统、肺和胃肠道。如果存在外周血嗜酸性粒细胞增多且病因不明,则怀疑患有该病。主要治疗方法是使用皮质类固醇。对皮质类固醇无反应的患者可使用伊马替尼、免疫调节药物、骨髓抑制疗法或美泊利珠单抗进行治疗。对于对其他疗法无反应的严重病例,可考虑使用阿仑单抗。在本文中,我们描述了一例主要累及心脏系统并形成左心室血栓的特发性高嗜酸性粒细胞综合征病例,该患者在服用华法林且国际标准化比值处于治疗范围内时并发了脑栓塞。我们的患者对类固醇反应良好,临床症状改善且嗜酸性粒细胞计数下降。

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