Objective: To investigate the clinical features of adult-onset chronic active Epstein-Barr virus infection (CAEBV). Methods: A total of 21 adult patients with CAEBV who were admitted to the department of General Internal Medicine at Peking Union Medical College Hospital from January 2006 to January 2016 were retrospectively analyzed. Demographic data, disease duration, clinical manifestations, laboratory findings, treatments and prognosis were reviewed. Results: Eighteen females and 3 males were enrolled with a mean age of 39 years. The most common clinical manifestations included fever in 20 patients, splenomegaly in 20 patients, lymphadenopathy in 18 patients, and hepatomegaly in 10 patients, followed by laryngopharyngeal disorders in 6 patients, pleural effusion and peritoneal effusion each in 5 patients, rash in 4 patients, interstitial lung disease in 3 patients, gastrointestinal hemorrhage in 2 patients, and peripheral neuropathy and pulmonary hypertension each in 1 patient. Six patients were complicated with hemophagocytic lymphohis-tioncytosis(HLH) that developed 5-17 (mean: 9) months following CAEBV onset, all of whom experienced hyperpyrexia, pancytopenia, lymphadenopathy, splenomegaly, and liver dysfunction, 3 with hepatomegaly. Nineteen of the 21 patients had received steroid therapy including 10 combined with immunosuppressive agents, 11 with antiviral therapy, and 8 with intravenous immunoglobulin. Thirteen patients died, including 10 of multiple organ failure, (including 6 of HLH) 2 of severe pulmonary infection, and 1 of lymphoma. Six patients remained on follow-up, yet 2 were missing. Conclusions: CAEBV is expected with severe condition and poor prognosis, which is likely to be complicated with HLH. Clinical physicians should pay attention to adult patients with fever, hepatosplenomegaly and lymphadenopathy, which suggests possible CAEBV.
目的： 探讨成人慢性活动性EB病毒感染（chronic active Epstein-Barr virus infection, CAEBV）的临床特征，为临床诊治提供依据。 方法： 回顾性分析2006年1月至2016年1月于北京协和医院普通内科住院的成人CAEBV患者共21例。 结果： 患者年龄（39.0±16.7）岁，男女比例1∶6。17例（81.0%）以发热为首发症状。最常见临床表现包括发热（20例）、脾脏肿大（20例）、淋巴结肿大（18例）、肝脏肿大（10例），其次为咽喉部病变（6例）、胸腔积液和腹腔积液（各5例）、皮疹（4例）、肺间质病变（3例）、消化道出血（2例）、周围神经病变和肺动脉高压（各1例）。18例出现血白细胞减低，17例贫血，15例血小板减低，15例出现肝功能异常。有6例患者合并噬血细胞性淋巴组织细胞增生症（hemophagocytic lymphohistiocytosis, HLH），发生于CAEBV起病后5~17个月，平均9个月，临床表现为高热、全血细胞减少、淋巴结肿大、脾脏肿大、肝功能损害，3例合并肝脏肿大。21例患者中，19例患者接受过糖皮质激素治疗，其中10例联合免疫抑制剂，11例接受过抗病毒治疗，8例给予静脉丙种球蛋白治疗。13例患者死亡，死亡原因包括多器官功能衰竭10例（其中HLH 6例）、重症肺部感染2例、淋巴瘤1例；6例仍在随访中，2例失访。 结论： 成人CAEBV临床表现复杂，易合并HLH，病情重，预后差，临床医生应早期识别和重视该病，对于不明原因发热、伴有肝脾肿大和淋巴结肿大的成人患者，应警惕CAEBV可能。.
Keywords: Adult; Chronic active Epstein-Barr virus infection; Epstein-Barr virus.