Chronic active Epstein–Barr virus (CAEBV) infection is a type of lymphoproliferative disorder characterized by chronic or recurrent infectious mononucleosis (IM)-like symptoms, which can have less-frequent clinical presentations. The prognosis of CAEBV is poor, and hematopoietic stem cell transplantation (HSCT) has been shown to be the only potentially effective treatment. In this article, we present a special CAEBV case of a patient who had no typical IM-like symptoms at the early stage, but manifested with severe and progressive coronary artery aneurysm (CAA), abdominal aortic lesions, and severe uveitis. These manifestations were uncommon features and could only be blocked by HSCT. A 4-year-old girl with no special medical history complained of decreased vision for 10 months and cough after physical activities for three months. The blurred vision grew rapidly worse within one month, until only light perception remained. She was diagnosed with uveitis and cataract, and received prednisone and ciclosporin A treatment. However, her vision did not improve. Physical examination showed slight hepatosplenomegaly. Ultrasonic cardiogram showed bilateral CAA (5.0 mm and 5.7 mm for inner diameters), and abdominal CT scan revealed a thickened aortic wall, as well as stenosis and dilation of the segmental abdominal aorta. Other significant findings were increased EBV-DNA (3.29 × 104 copies/mL) from peripheral blood, positive EBV antibodies (EBV-CA-IgG, EBV-EA-IgA, and EBV-NA-IgG), and positive EBV-encoded small RNAs found by bone marrow biopsy. Based on her clinical manifestations and evidence for EBV infection, we diagnosed CAEBV. She received allogeneic HSCT, and the cataract operation was performed after HSCT. EBV-DNA could not be detected in peripheral blood after HSCT. Her CAAs did not progress, and uveitis was well controlled. Her vision recovered gradually over the 3 years after HSCT. We present a rare CAEBV case of a patient who suffered from uncommon and severe cardiovascular and ocular involvement that was relieved by HSCT. Therefore, early recognition and diagnosis of CAEBV are of vital importance to improve its prognosis. In summary, this atypical CAEBV case could help us recognize similar cases more easily, make the right diagnosis as early as possible, and deliver proper and timely treatment.

中文翻译：

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慢性活动性爱泼斯坦-巴尔病毒感染表现为冠状动脉瘤和葡萄膜炎

慢性活动性爱泼斯坦-巴尔病毒 (CAEBV) 感染是一种淋巴组织增生性疾病，其特征是慢性或复发性传染性单核细胞增多症 (IM) 样症状，临床表现可能较少。CAEBV 的预后很差，造血干细胞移植 (HSCT) 已被证明是唯一可能有效的治疗方法。在本文中，我们介绍了一个特殊的 CAEBV 病例，该患者早期没有典型的 IM 样症状，但表现为严重和进行性冠状动脉瘤 (CAA)、腹主动脉病变和严重的葡萄膜炎。这些表现是不常见的特征，只能通过 HSCT 来阻止。一名无特殊病史的 4 岁女孩主诉视力下降 10 个月，体力活动后咳嗽 3 个月。1个月内视力模糊迅速恶化，直到只剩下光感。她被诊断出患有葡萄膜炎和白内障，并接受了泼尼松和环孢素 A 治疗。然而，她的视力并没有改善。体格检查显示轻度肝脾肿大。超声心动图显示双侧 CAA（内径为 5.0 mm 和 5.7 mm），腹部 CT 扫描显示主动脉壁增厚，以及节段性腹主动脉狭窄和扩张。其他重要发现包括来自外周血的 EBV-DNA（3.29 × 104 拷贝/mL）、阳性 EBV 抗体（EBV-CA-IgG、EBV-EA-IgA 和 EBV-NA-IgG）和阳性 EBV 编码的小骨髓活检发现的 RNA。根据她的临床表现和 EBV 感染证据，我们诊断为 CAEBV。她接受了异基因造血干细胞移植，HSCT后进行白内障手术。HSCT后外周血中未检测到EBV-DNA。她的 CAA 没有进展，葡萄膜炎得到了很好的控制。在 HSCT 后的 3 年里，她的视力逐渐恢复。我们介绍了一个罕见的 CAEBV 病例，该病例患有罕见且严重的心血管和眼部受累，但通过 HSCT 得到缓解。因此，CAEBV的早期识别和诊断对于改善其预后至关重要。综上所述，这个非典型CAEBV病例可以帮助我们更轻松地识别类似病例，尽早做出正确诊断，并提供适当及时的治疗。在 HSCT 后的 3 年里，她的视力逐渐恢复。我们介绍了一个罕见的 CAEBV 病例，该病例患有罕见且严重的心血管和眼部受累，但通过 HSCT 得到缓解。因此，CAEBV的早期识别和诊断对于改善其预后至关重要。综上所述，这个非典型CAEBV病例可以帮助我们更轻松地识别类似病例，尽早做出正确诊断，并提供适当及时的治疗。在 HSCT 后的 3 年里，她的视力逐渐恢复。我们介绍了一个罕见的 CAEBV 病例，该病例患有罕见且严重的心血管和眼部受累，但通过 HSCT 得到缓解。因此，CAEBV的早期识别和诊断对于改善其预后至关重要。综上所述，这个非典型CAEBV病例可以帮助我们更轻松地识别类似病例，尽早做出正确诊断，并提供适当及时的治疗。