CAJ | 학술논문

目的报告12例符合诊断标准的严重慢性活动性EB病毒感染(SCAEBV).方法回顾性分析12例SCAEBV患儿的临床资料、实验室、血清学、影像学和病理学检查以及初步随访的结果.结果 SCAEBV临床主要表现为发热、脾肿大、肝肿大、淋巴结肿大,其他表现有皮疹、黄疸、肺动脉高压、口腔溃疡和胆囊炎等.辅助检查的异常包括乳酸脱氢酶升高、肝功能异常、贫血、白细胞降低、中性粒细胞降低、血小板减少以及胸部X线的异常等.所有患者均有抗EB病毒衣壳抗原IgG抗体的升高,而其IgM和IgA的阳性率分别为33.3%和66.7%;检测5例EB病毒早期抗原IgG均为阳性,而其IgA的阳性率为40.0%.实时定量聚合酶链反应检测外周血EB病毒DNA载量(中位数)为8.12×10~6copies/ml.12例患者中4例死亡,其中2例死于噬血细胞综合征,1例死于多重感染,1例死于多脏器功能衰竭;其余病例随访中1例发生了T细胞性非霍奇金淋巴瘤,1例脾切除术后发生了肝肺综合征.结论 SCAEBV临床表现多样,常伴有各系统严重并发症,预后差,死亡率高,应引起儿科临床医师的关注.
목적 보고12례부합진단표준적엄중만성활동성EB병독감염(SCAEBV).방법 회고성분석12례SCAEBV환인적림상자료、실험실、혈청학、영상학화병이학검사이급초보수방적결과.결과 SCAEBV림상주요표현위발열、비종대、간종대、림파결종대,기타표현유피진、황달、폐동맥고압、구강궤양화담낭염등.보조검사적이상포괄유산탈경매승고、간공능이상、빈혈、백세포강저、중성립세포강저、혈소판감소이급흉부X선적이상등.소유환자균유항EB병독의각항원IgG항체적승고,이기IgM화IgA적양성솔분별위33.3%화66.7%;검측5례EB병독조기항원IgG균위양성,이기IgA적양성솔위40.0%.실시정량취합매련반응검측외주혈EB병독DNA재량(중위수)위8.12×10~6copies/ml.12례환자중4례사망,기중2례사우서혈세포종합정,1례사우다중감염,1례사우다장기공능쇠갈;기여병례수방중1례발생료T세포성비곽기금림파류,1례비절제술후발생료간폐종합정.결론 SCAEBV림상표현다양,상반유각계통엄중병발증,예후차,사망솔고,응인기인과림상의사적관주.
Objective There are two different types of chronic active Epstein-Barr virus (CAEBV) infection: chronic EBV (CEBV) having persistent infectious mononucleosis (IM)-like illness with relatively good prognosis, and severe CAEBV (SCAEBV)infection that has rather severe manifestations and generally poor prognosis with many life-threatening complications, such as EBV-associated malignant lymphoma and hemophagocytic syndrome (HPS). The purpose of this study was to clarify the clinical and prognostic characteristics in 12 cases with SCAEBV infection. Method Data of 12 cases with SCAEBV infection were analyzed retrospectively, which included clinical and auxiliary examination, pathological data, especially EB virus (EBV)-antibodies and DNA in peripheral blood mononuclear cells (PBMC) and infected tissue, and follow-up information. Result Of the 12 cases, 7 were male and 5 were female. The age at the onset of diseases ranged from 35 months to 14 years (median, 11 years). The major manifestations were fever (100%), splenomegaly (91.7%), hepatomegaly (83.3%), lymphadenopathy (75.0%), and others, including skin rash, development retardation, jaundice, ascites, pulmonary hypertension, oral ulcer, cholecystitis and pleural effusion. The abnormalities of auxiliary examination were as follows: elevated LDH level (91.7%), liver dysfunction (83.3%), anemia (75.0%), leukopenia (58.3%), neutropenia (50.0%), thrombocytopenia (25.0%) and abnormal chest X-ray findings. At the time of onset, 58.3% of the patients had an IM-like illness. In all of the 12 cases, EBV serologic tests revealed high IgG antibody levels against EB viral capsid antigen (VCA). The patients often had positive IgM and IgA antibodies against VCA (33.3% and 66.7%) as well. Elevated IgG antibody level to early antigen (EA) (100.0%), occasionally positive IgA antibody (40.0%) were also seen. The mean load of EBV-DNA detected by real-time polymerase chain reaction (PCR) in the PBMC was(8. 12×10~6, median)copies/ml.Four of 12 cases presented a poor clinical course, two of whom died from EBV-associated HPS, 1 from severe multiple pathogens infection, and 1 from multiple organ failure. In addition, 1 case developed Hodgkin's T cell lymphoma and another case showed hepatopulmonary syndrome in 2 years after splenectomy. Conclusions The clinical feature of SCAEBV infection varied exceedingly. EBV-DNA load in PBMC of SCAEBV infected patients was significantly increased. More attention should be paid to the disease because of its severe comphcations, poor prognosis and high mortality.