Yan Jianghong, Ren Huiqiang, Wang Qing, Zhao Linmeng, Zhai Xiaoying
The Pediatric Research Institute, Hebei Children's Hospital Shijiazhuang, Hebei, China.
Department of Pathology, Hebei Children's Hospital Shijiazhuang, Hebei, China.
Am J Transl Res. 2025 Jul 15;17(7):5207-5213. doi: 10.62347/FSWU8553. eCollection 2025.
Chronic active Epstein-Barr virus (CAEBV) infection is a rare and fatal disease caused by persistent EBV infection. During the progressive phase of CAEBV, children are highly susceptible to life-threatening complications such as hemophagocytic syndrome, multiple organ failure (particularly liver and kidney failure), coagulation disorders, and hemangiomas, resulting in a high mortality rate. Traditional treatments such as immunotherapy or chemotherapy are limited in effectiveness, and children may succumb to the disease within a few years. Currently, allogeneic therapy (hematopoietic stem cell transplantation [HSCT]) is the only curative measure for this disease. However, this procedure may lead to a series of complications that threaten the lives of the transplanted children. Therefore, mastering the correct management approach and exploring new preventive and therapeutic measures are crucial. This paper reports a case of a child with CAEBV who developed complications after allogeneic HSCT. Through the comprehensive use of immunomodulators and personalized treatment strategies, patient's conditions was improved significantly.
慢性活动性EB病毒(CAEBV)感染是一种由持续性EB病毒感染引起的罕见致命疾病。在CAEBV的进展期,儿童极易出现危及生命的并发症,如噬血细胞综合征、多器官衰竭(尤其是肝衰竭和肾衰竭)、凝血障碍和血管瘤,导致死亡率很高。免疫疗法或化疗等传统治疗方法效果有限,儿童可能在几年内死于该病。目前,同种异体疗法(造血干细胞移植[HSCT])是治疗这种疾病的唯一治愈性措施。然而,该手术可能会引发一系列威胁移植儿童生命的并发症。因此,掌握正确的管理方法并探索新的预防和治疗措施至关重要。本文报告了1例接受同种异体HSCT后出现并发症的CAEBV患儿。通过综合使用免疫调节剂和个性化治疗策略,患儿病情得到显著改善。