摘要慢性活动性EB病毒感染(CAEBV)主要表现为慢性或复发性传染性单核细胞增多症样症状,包括发热、肝脾大、肝功能异常、淋巴结病、蚊虫叮咬过敏、皮疹等;伴外周血高EB病毒(EBV)载量和/或异常的EBV相关抗体.该病临床少见,但预后较差,发病机制目前尚不清楚.传统的抗病毒治疗无效,免疫抑制剂、免疫调节治疗、免疫细胞治疗等措施效果有限或仅短期暂时有效,异基因造血干细胞移植是治愈该病的有效方法,但也存在一些风险.

abstractsChronic active Epstein-Barr virus (EBV) infection (CAEBV) is characterized by chronic or recurrent infectious mononucleosis-like symptoms,such as fever,hepatomegaly,splenomegaly,liver dysfunction,lymphadenopathy,hypersensitivity to mosquito bites,skin rash.Patients with CAEBV have high viral loads in their peripheral blood and/or an unusual pattern of EBV-related antibodies.This disease is rare but severe with high mortality,the pathogenesis is unknown though there is accumulating evidence that the clonal expansion of EBV-infected T or natural killer (NK) cells plays a central role in the pathogenesis of CAEBV.Because antiviral therapy is generally ineffective for this disease,numerous agents have been tried for the treatment of CAEBV,e.g.immunosuppressive agents,immunomodulatory therapy,immune cell therapy.Allogeneic hematopoietic stem cell transplants have been reported to be successful,but there still has some risks.