Kawamoto Keisuke, Miyoshi Hiroaki, Seto Masao, Kimura Hiroshi, Ohshima Koichi
Department of Hematology, Endocrinology, and Metabolism, Faculty of Medicine, Niigata University.
Department of Pathology, Kurume University School of Medicine.
Rinsho Ketsueki. 2019;60(8):944-952. doi: 10.11406/rinketsu.60.944.
Chronic active Epstein-Barr virus infection (CAEBV) presents with mononucleosis-like symptoms such as chronic persistent or recurrent pyrexia, lymphadenopathy, and hepatosplenomegaly because of the reactivation of Epstein-Barr virus (EBV) as demonstrated by the recurrence of EBV-infected cells. The mechanism of CAEBV remains obscure, and CAEBV can lead to fatal conditions such as hemophagocytic syndrome and malignant lymphoma by clonal expansion of EBV-infected T- or NK-cells. Without hematopoietic stem cell transplantation, CAEBV has a poor prognosis. CAEBV is listed in the revised 2016 World Health Organization classification as a chronic active EBV infection of T- and NK-cell types, systemic form, among EBV-positive T- and NK-cell lymphoproliferative diseases of childhood. However, similar clinical conditions have been reported in adult patients. Therefore, we investigated the clinical features of adult patients with CAEBV-like features (adult-onset CAEBV) in a relatively small number of cases. Additionally, genetic alterations related to CAEBV development have also been reported. Along with these results, we reviewed the clinical characteristics of adult-onset CAEBV.
慢性活动性EB病毒感染（CAEBV）表现为类似传染性单核细胞增多症的症状，如慢性持续性或复发性发热、淋巴结病和肝脾肿大，这是由于EB病毒（EBV）重新激活所致，EBV感染细胞的复发可证明这一点。CAEBV的发病机制仍不清楚，并且CAEBV可通过EBV感染的T细胞或NK细胞的克隆性扩增导致诸如噬血细胞综合征和恶性淋巴瘤等致命疾病。若无造血干细胞移植，CAEBV的预后较差。在2016年世界卫生组织修订分类中，CAEBV被列为儿童EBV阳性T细胞和NK细胞淋巴增殖性疾病中T细胞和NK细胞类型的慢性活动性EBV感染，全身型。然而，成人患者中也报告了类似的临床情况。因此，我们在相对少数的病例中研究了具有CAEBV样特征的成人患者（成人发病的CAEBV）的临床特征。此外，也有关于与CAEBV发生相关的基因改变的报道。结合这些结果，我们回顾了成人发病的CAEBV的临床特征。
