Clinical characteristics of 111 patients with chronic active Epstein-Barr virus infection / 中华传染病杂志

ABSTRACT

Objective:To analyze the clinical features of chronic active Epstein-Barr virus infection (CAEBV) in order to reduce the rates of underdiagnosis and misdiagnosis of this disease.Methods:The CAEBV related literatures of PubMed, Embase, Cochrane Library, China National Knowledge Infrastructure, WanFang Database and Chongqing VIP since the first literature published (May 1987) until August 29, 2020 were searched. The clinical characteristics, laboratory examinations, outcome and causes of death of CAEBV patients were retrospectively analyzed. Statistical analysis was performed by Mann-Whitney U test, chi-square test or Fisher′s exact probability test. Results:A total of 111 patients aged 22.0 (10.0, 39.0) years were included from 46 articles. There were 64 cases (57.7%) in the age ≥18 years group and 47 cases (42.3%) in the age <18 years group. Fever, splenomegaly, hepatomegaly, and lymph node enlargement were common clinical manifestations, with incidences of 95.5%(106/111), 84.7%(94/111), 57.7%(64/111) and 56.8%(63/111), respectively. The incidences of rash and hepatomegaly in the age ≥18 years group were 3.1%(2/64) and 45.3%(29/64), respectively, which were both lower than those in patients aged <18 years group (27.7%(13/47) and 74.5%(35/47), respectively), while the incidence of abnormal liver biochemical indexes was higher (45.3%(29/64) vs 23.4%(11/47)). The differences were all statistically significant ( χ2=13.957, 9.436 and 5.643, respectively, all P<0.05). Of the 70 patients with follow-up outcomes, 38(54.3%) died and 32(45.7%) survived. The causes of death included gastrointestinal bleeding, severe infection, respiratory failure, liver failure, etc. The incidences of splenomegaly in the death and survival groups were 92.1%(35/38) and 68.8%(22/32), respectively. The difference was statistically significant ( χ2=6.266, P<0.05). Of 21 death and 17 survival cases in the age <18 years group, 15(71.4%) and two cases were combined hemophagocytic lymphohistiocytosis (HLH), respectively, with statistical significance ( χ2=13.527, P<0.01). Of the 90 patients whose HLH-related information was available, 38(42.2%) combined HLH and 52(57.8%) without HLH, with 36.8%(14/38) and 65.4% (34/52) of males, respectively. The difference of gender distribution was statistically significant ( χ2=7.187, P=0.007). The treatment regimens of the 111 CAEBV patients during the course of disease were various, but the detailed information was lacking. Conclusions:The clinical manifestations of CAEBV are diverse. CAEBV can be complicated with fatal complications, lacks of effective treatment, and shows poor prognosis. It is necessary to actively carry out related research to improve the understanding of the disease, and explore effective treatment and reduce mortality.