Si Jinping, Zhang Xinxin, Chen Na, Sun Fangfang, Du Ping, Li Zhiyong, Tian Di, Sun Xiuli, Sun Guozhen, Cong Tao, Du Xuemei, Liu Ying
Department of Cardiology, First Affiliated Hospital of Dalian Medical University, Dalian, China.
Department of Nuclear Medicine, First Affiliated Hospital of Dalian Medical University, Dalian, China.
Front Cardiovasc Med. 2022 Jun 3;9:903323. doi: 10.3389/fcvm.2022.903323. eCollection 2022.
Eosinophilic leukemia (EL) is a rare, serious and potentially life-threatening condition characterized by the overproduction of eosinophils leading to tissue eosinophilic infiltration and damage. Although multiple organ systems may be involved, progressive eosinophilic myocarditis (EM) is the most common cause of morbidity and mortality. Early diagnosis and follow-up surveillance combined with multimodal imaging are crucial for appropriate treatment of EM.
It's a rare case of EL with EM and intracardiac thrombus in a 59-year-old patient who presented with asthenia for 3 weeks. Full blood count analysis indicated significant eosinophilia. Bone marrow aspirate revealed dysplastic eosinophilia and a FIP1L1-PDGFRA fusion gene (4q12) was detected, confirming EL. Echocardiography revealed EM with intracardiac thrombus. This was later confirmed by cardiac magnetic resonance imaging. The patient was commenced on imatinib and prednisolone and good clinical response was obtained. Through 18F-FAPI PET/CT imaging, we obtained visualization of fibroblast activation changes in the early stage of cardiac structure remodeling. With anti-fibrotic therapy after heart failure, the patient achieved a good clinical response.
This case demonstrates visualization of fibroblast activation after EM. Multimodality imaging can provide early diagnosis and may guide tailored antifibrotic therapy in early stage of EM.
嗜酸性粒细胞白血病（EL）是一种罕见、严重且可能危及生命的疾病，其特征是嗜酸性粒细胞过度生成，导致组织嗜酸性粒细胞浸润和损伤。尽管多个器官系统可能受累，但进行性嗜酸性粒细胞性心肌炎（EM）是发病和死亡的最常见原因。早期诊断、随访监测以及多模态成像对于EM的恰当治疗至关重要。
这是一例罕见的59岁EL合并EM及心内血栓的病例，患者出现乏力3周。全血细胞计数分析显示显著的嗜酸性粒细胞增多。骨髓穿刺显示发育异常的嗜酸性粒细胞增多，检测到FIP1L1-PDGFRA融合基因（4q12），确诊为EL。超声心动图显示EM合并心内血栓。随后心脏磁共振成像证实了这一情况。患者开始使用伊马替尼和泼尼松龙治疗，获得了良好的临床反应。通过18F-FAPI PET/CT成像，我们观察到心脏结构重塑早期成纤维细胞激活的变化。心力衰竭后给予抗纤维化治疗，患者获得了良好的临床反应。
本病例展示了EM后成纤维细胞激活的影像学表现。多模态成像可提供早期诊断，并可能在EM早期指导针对性的抗纤维化治疗。
