Chao Bo H, Cline-Parhamovich Karen, Grizzard John D, Smith Thomas J
Department of Internal Medicine, Medical College of Virginia, Virginia Commonwealth University, Richmond, Virginia, USA.
Am J Hematol. 2007 Oct;82(10):920-3. doi: 10.1002/ajh.20933.
Hypereosinophilic syndrome (HES) is a rare disorder that can manifest in various organ systems. We report the case of a 54-year-old woman with a remote history of seizure disorder who presented with early signs of right-sided heart failure. Laboratory studies showed significant eosinophilia (8 x 10(9) l(-1)). Computed tomography showed heterogeneity of the liver, mild ascites, moderate pleural effusion, multiple small pulmonary emboli, and a large right ventricular mass. Cardiac magnetic resonance imaging demonstrated that the right ventricular mass was due to thrombus and extensive endomyocardial fibrosis, consistent with Loeffler's endocarditis. Bone marrow biopsy showed marked eosinophilia but no abnormal myeloid maturation or a lymphoproliferative disorder; flow cytometry showed no clonality. Extensive infectious, immunologic, and toxicological studies were negative. Despite resolution of peripheral eosinophilia with medical management, including corticosteroids and cytotoxic agents, anticoagulation for pulmonary emboli and ventricular thrombus, and conventional treatment for heart failure, she developed worsening anasarca and died from ventricular fibrillation within 4 weeks of presentation. Autopsy confirmed the diagnosis. Loeffler's endocarditis, usually a late manifestation of HES, is characterized by fibrous thickening of the endocardium, leading to apical obliteration and restrictive cardiomyopathy, resulting in heart failure, thromboembolic events, or atrial fibrillation. HES is a potentially fatal disease with less than 50% reported 10-year survival. This case presentation is unusual in its rapidly progressive course leading to sudden death.
高嗜酸性粒细胞综合征（HES）是一种罕见疾病，可累及多个器官系统。我们报告了一例54岁女性患者，有癫痫病史，出现了右侧心力衰竭的早期症状。实验室检查显示显著嗜酸性粒细胞增多（8×10⁹/L）。计算机断层扫描显示肝脏不均质、轻度腹水、中度胸腔积液、多发小的肺栓塞以及一个大的右心室肿块。心脏磁共振成像显示右心室肿块是由血栓和广泛的心内膜纤维化所致，符合勒夫勒心内膜炎。骨髓活检显示显著嗜酸性粒细胞增多，但无异常髓系成熟或淋巴增殖性疾病；流式细胞术显示无克隆性。广泛的感染、免疫和毒理学检查均为阴性。尽管通过包括皮质类固醇和细胞毒性药物在内的药物治疗使外周嗜酸性粒细胞增多得到缓解，对肺栓塞和心室血栓进行了抗凝治疗，并对心力衰竭进行了常规治疗，但她仍出现了进行性加重的全身性水肿，并在就诊后4周内因心室颤动死亡。尸检确诊了诊断。勒夫勒心内膜炎通常是HES的晚期表现，其特征为心内膜纤维性增厚，导致心尖闭塞和限制性心肌病，进而引起心力衰竭、血栓栓塞事件或心房颤动。HES是一种潜在致命性疾病，据报道10年生存率低于50%。该病例表现的不寻常之处在于其迅速进展的病程导致猝死。
