Department of Infectious Diseases, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China.
Department of Hematology and Oncology, Affiliated Bayi Children's Hospital, The Seventh Medical Center of PLA General Hospital, Beijing, China.
Virol J. 2020 Oct 29;17(1):166. doi: 10.1186/s12985-020-01409-8.
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 × 10 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.
慢性活动性 EBV（CAEBV）感染是一种淋巴组织增生性疾病，其特征为慢性或复发性传染性单核细胞增多症（IM）样症状，且临床表现可能不典型。CAEBV 的预后较差，造血干细胞移植（HSCT）已被证明是唯一可能有效的治疗方法。本文介绍了一例特殊的 CAEBV 病例，该患者在早期无典型 IM 样症状，但表现为严重且进行性的冠状动脉瘤（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 来阻断。
一名 4 岁女孩，无特殊病史，主诉 10 个月视力下降，3 个月后活动后咳嗽。1 个月内视力迅速恶化，直至仅能感知光。她被诊断为葡萄膜炎和白内障，并接受了泼尼松和环孢素 A 治疗，但视力没有改善。体格检查显示轻度肝脾肿大。超声心动图显示双侧 CAA（内径分别为 5.0mm 和 5.7mm），腹部 CT 扫描显示主动脉壁增厚，以及腹主动脉节段性狭窄和扩张。其他重要发现包括外周血 EBV-DNA 升高（3.29×10 拷贝/ml）、EBV 抗体阳性（EBV-CA-IgG、EBV-EA-IgA 和 EBV-NA-IgG），以及骨髓活检发现 EBV 编码的小 RNA 阳性。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 HSCT，HSCT 后进行了白内障手术。HSCT 后外周血中无法检测到 EBV-DNA。她的 CAA 没有进展，葡萄膜炎得到了很好的控制。HSCT 后 3 年内，她的视力逐渐恢复。
我们报告了一例罕见的 CAEBV 病例，该患者患有罕见且严重的心血管和眼部受累，HSCT 缓解了这些受累。因此，早期识别和诊断 CAEBV 对于改善其预后至关重要。总之，这个非典型的 CAEBV 病例可以帮助我们更容易地识别类似病例，尽早做出正确诊断，并给予适当和及时的治疗。
