Kimura Hiroshi, Cohen Jeffrey I
Department of Virology, Nagoya University Graduate School of Medicine, Nagoya, Japan.
Medical Virology Section, Laboratory of Infectious Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, United States.
Front Immunol. 2017 Dec 22;8:1867. doi: 10.3389/fimmu.2017.01867. eCollection 2017.
Chronic active Epstein-Barr virus (CAEBV) disease is a rare disorder in which persons are unable to control infection with the virus. The disease is progressive with markedly elevated levels of EBV DNA in the blood and infiltration of organs by EBV-positive lymphocytes. Patients often present with fever, lymphadenopathy, splenomegaly, EBV hepatitis, or pancytopenia. Over time, these patients develop progressive immunodeficiency and if not treated, succumb to opportunistic infections, hemophagocytosis, multiorgan failure, or EBV-positive lymphomas. Patients with CAEBV in the United States most often present with disease involving B or T cells, while in Asia, the disease usually involves T or NK cells. The only proven effective treatment for the disease is hematopoietic stem cell transplantation. Current studies to find a cause of this disease focus on immune defects and genetic abnormalities associated with the disease.
慢性活动性EB病毒（CAEBV）病是一种罕见的疾病，患者无法控制该病毒的感染。该病呈进行性发展，血液中EBV DNA水平显著升高，EBV阳性淋巴细胞浸润器官。患者常表现为发热、淋巴结病、脾肿大、EBV肝炎或全血细胞减少。随着时间的推移，这些患者会出现进行性免疫缺陷，若不治疗，会死于机会性感染、噬血细胞综合征、多器官衰竭或EBV阳性淋巴瘤。美国的CAEBV患者最常表现为涉及B细胞或T细胞的疾病，而在亚洲，该病通常涉及T细胞或NK细胞。该病唯一经证实有效的治疗方法是造血干细胞移植。目前寻找该病病因的研究集中在与该病相关的免疫缺陷和基因异常上。
