Gotoh Kensei, Ito Yoshinori, Shibata-Watanabe Yukiko, Kawada Jun-ichi, Takahashi Yoshiyuki, Yagasaki Hiroshi, Kojima Seiji, Nishiyama Yukihiro, Kimura Hiroshi
Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan.
Clin Infect Dis. 2008 May 15;46(10):1525-34. doi: 10.1086/587671.
Chronic active Epstein-Barr virus (EBV) infection is characterized by recurrent infectious mononucleosis-like symptoms, and infected patients have high viral loads in their peripheral blood. Standard therapy for the disease has not yet been established. Recently, hematopoietic stem cell transplantation (HSCT) has been introduced and has the potential to become a standard treatment, although guidelines for HSCT to treat chronic active EBV infection have not yet been proposed.
Fifteen patients were retrospectively analyzed, both clinically and virologically, to investigate the factors associated with prognosis of chronic active EBV infection treated with HSCT.
After HSCT, 7 patients died after survival periods that ranged from 1 to 16 months (mean duration of survival after HSCT, 5 months). Three patients were considered to have died of transplantation-related complications. The duration between infection onset and diagnosis was significantly longer in patients who died than in those who survived. Five of the 7 patients who died experienced > or =3 life-threatening complications. The plasma concentrations of interferon-gamma, interleukin-10, thrombomodulin, and soluble E-selectin did not differ significantly between the groups of patients. With regard to sequence variations in the EBV latent membrane protein 1 gene, no specific patterns were found in the patients who died. Importantly, the plasma EBV load at diagnosis was significantly higher in patients who died than in living patients. Moreover, plasma viral load was shown to be an important factor to monitor during follow-up for patients after HSCT.
The number of life-threatening complications and plasma viral load are indicative of the stage of disease progression and may be useful factors for predicting the outcome of HSCT.
慢性活动性EB病毒（EBV）感染的特征为反复出现传染性单核细胞增多症样症状，且感染患者外周血中的病毒载量较高。该疾病的标准治疗方法尚未确立。最近，造血干细胞移植（HSCT）已被引入，并且有潜力成为标准治疗方法，尽管尚未提出用于治疗慢性活动性EBV感染的HSCT指南。
对15例患者进行回顾性临床和病毒学分析，以研究与HSCT治疗慢性活动性EBV感染预后相关的因素。
HSCT后，7例患者在1至16个月的生存期后死亡（HSCT后的平均生存时间为5个月）。3例患者被认为死于移植相关并发症。死亡患者从感染发作到诊断的时间明显长于存活患者。7例死亡患者中有5例经历了≥3次危及生命的并发症。患者组间干扰素-γ、白细胞介素-10、血栓调节蛋白和可溶性E选择素的血浆浓度无显著差异。关于EBV潜伏膜蛋白1基因的序列变异，在死亡患者中未发现特定模式。重要的是，死亡患者诊断时的血浆EBV载量明显高于存活患者。此外，血浆病毒载量被证明是HSCT后患者随访期间需要监测的重要因素。
危及生命的并发症数量和血浆病毒载量可指示疾病进展阶段，可能是预测HSCT结果的有用因素。
