Agudo Margarida, Santos Francisca, Teixeira Reis Ana, Moura Pedro, Marques Susana
Internal Medicine, Centro Hospitalar de Setúbal, Setúbal, PRT.
Medicine, Centro Hospitalar Do Médio Ave, Vila Nova de Famalicao, PRT.
Cureus. 2023 Jun 5;15(6):e39964. doi: 10.7759/cureus.39964. eCollection 2023 Jun.
Idiopathic hypereosinophilic syndrome is a rare condition characterized by persistent severe eosinophilia and organ damage without any apparent cause. A 20-year-old male patient with no significant medical history was admitted to the Emergency Department with retrosternal chest pain, fatigue and asthenia. EKG showed ST elevation I, II, III, aVF, V4-V6 and blood tests showed elevated troponin levels. An echocardiogram was performed revealing severe global left ventricular systolic dysfunction. Further evaluations included cardiac magnetic resonance imaging and endomyocardial biopsy, confirming the diagnosis of eosinophilic myocarditis. The patient was started on systemic corticosteroid therapy, resulting in clinical improvement. The patient was discharged after 12 days of hospitalization, following a recovery of biventricular function and he was told to continue oral corticosteroid therapy at home. Further investigation ruled out other causes of hypereosinophilic syndromes, therefore the diagnosis of idiopathic hypereosinophilic syndrome was assumed. Despite the attempt to reduce corticosteroid therapy, the eosinophil count became elevated again, so the dosage was increased and associated with azathioprine with subsequent favorable analytical evolution. This case highlights the challenges in diagnosing and managing idiopathic hypereosinophilic syndrome and emphasizes the importance of prompt treatment initiation to prevent complications.
特发性嗜酸性粒细胞增多综合征是一种罕见疾病,其特征为持续性严重嗜酸性粒细胞增多和无明显病因的器官损害。一名无显著病史的20岁男性患者因胸骨后胸痛、乏力和疲倦入住急诊科。心电图显示I、II、III、aVF、V4 - V6导联ST段抬高,血液检查显示肌钙蛋白水平升高。超声心动图显示严重的全心左心室收缩功能障碍。进一步评估包括心脏磁共振成像和心内膜心肌活检,确诊为嗜酸性粒细胞性心肌炎。患者开始接受全身糖皮质激素治疗,临床症状改善。住院12天后,双心室功能恢复,患者出院,并被告知在家继续口服糖皮质激素治疗。进一步检查排除了嗜酸性粒细胞增多综合征的其他病因,因此诊断为特发性嗜酸性粒细胞增多综合征。尽管试图减少糖皮质激素治疗,但嗜酸性粒细胞计数再次升高,因此增加了剂量并联合使用硫唑嘌呤,随后分析结果呈有利进展。该病例突出了特发性嗜酸性粒细胞增多综合征诊断和管理中的挑战,并强调了及时开始治疗以预防并发症的重要性。