Division of Allergy, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
Division of Cardiology, Department of Medicine, Heart Vascular Stroke Institute, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
Yonsei Med J. 2023 Feb;64(2):148-151. doi: 10.3349/ymj.2022.0279.
Idiopathic hypereosinophilic syndrome (iHES) is a rare systemic disease that is characterized by persistent peripheral eosinophilia (absolute eosinophil count ≥1500/uL) for more than 6 months, with end-organ damage and absence of a primary cause for eosinophilia. Coronary artery aneurysm (CAA) is a rare but life-threatening complication. Here, we report a case of CAA with thrombosis in a patient with iHES in whom the disease activity was well-controlled (eosinophil count <500/uL) for several years. Despite modest control of the disease activity, giant CAA can be associated with iHES; and therefore, close surveillance and monitoring for the development of complications is warranted.
特发性嗜酸性粒细胞增多综合征(iHES)是一种罕见的系统性疾病,其特征为持续外周血嗜酸性粒细胞增多(绝对嗜酸性粒细胞计数≥1500/μL)超过 6 个月,伴有终末器官损害且无嗜酸性粒细胞增多的主要原因。冠状动脉瘤(CAA)是一种罕见但危及生命的并发症。在此,我们报告一例 iHES 伴血栓形成的 CAA 病例,该患者的疾病活动度在数年内得到了很好的控制(嗜酸性粒细胞计数<500/μL)。尽管疾病活动度得到了适度控制,但巨大的 CAA 仍可能与 iHES 相关;因此,需要密切监测和随访并发症的发生。