Fulminant Idiopathic Hypereosinophilic Syndrome Presenting with Cardiac, Neurologic and Haematologic Involvement

暴发性特发性嗜酸性粒细胞增多症伴心脏、神经和血液系统受累

阅读:1

Abstract

INTRODUCTION: Hypereosinophilic syndrome (HES) is a rare condition characterised by persistent peripheral eosinophilia and associated with multi-organ damage. The most common sources of both morbidity and mortality in HES patients relate to cardiac or neurologic complications, necessitating early recognition and intervention. CASE DESCRIPTION: A 44-year-old male was admitted with acute chest pain followed by progressive encephalopathy which worsened, indicating the need for intubation. Laboratory studies were significant for marked hypereosinophilia, massive troponin elevation and profound thrombocytopenia. Brain magnetic resonance imaging (MRI) revealed bilateral watershed infarcts. Despite normal ventricular function and mobility on echocardiography, the onset of peak troponin levels concurrently with eosinophilia, resolving promptly after treatment, led us to consider probable acute eosinophilic myocarditis (EM). Thorough workup excluded secondary (parasitic, autoimmune) clonal causes, thus fulfilling consensus-defined criteria for idiopathic HES. High-dose intravenous corticosteroids (methylprednisolone 1 g/day × 3 days) resulted in rapid, synchronous normalisation of eosinophilia, troponin levels and platelet counts, confirming a steroid-responsive, eosinophil-driven pathology. The patient was left with persistent severe neurologic deficits. CONCLUSION: This case highlights the destructive potential of acute eosinophil degranulation across multiple organs. The rapid improvement with corticosteroids underscores the importance of early recognition and immediate immunosuppressive therapy to limit organ damage. Clinicians should maintain high suspicion for HES in adults with unexplained eosinophilia and multi-organ involvement. LEARNING POINTS: Maintain suspicion for HES in unexplained eosinophilia with multi-organ dysfunction.Eosinophilic myocarditis may present with high troponin despite normal echocardiogram.Bilateral watershed infarcts reflect microvascular hypoperfusion due to eosinophilic endothelial injury.Thrombocytopenia often resolves rapidly with corticosteroids.Early immunosuppression prevents irreversible organ damage.

特别声明

1、本页面内容包含部分的内容是基于公开信息的合理引用;引用内容仅为补充信息,不代表本站立场。

2、若认为本页面引用内容涉及侵权,请及时与本站联系,我们将第一时间处理。

3、其他媒体/个人如需使用本页面原创内容,需注明“来源:[生知库]”并获得授权;使用引用内容的,需自行联系原作者获得许可。

4、投稿及合作请联系:info@biocloudy.com。