慢性活动性EB病毒感染的研究进展

慢性活动性EB病毒感染（Chmnic active EBV infection,CAEBV）是一种少见的发生在无明确免疫缺陷个体的综合征,临床表现多种多样,其病理改变几乎可涉及到各个器官。主要表现为EBV感染后出现慢性或复发性传单样症状,伴随EBV抗体的异常改变或病毒载量的升高,病程中可出现严重的或致死的并发症。文章就其发病机制、临床表现、实验室检查及诊治方案进展作一介绍,以提高临床对慢性活动性EBV的诊治水平。     

慢性活动性EB病毒感染的研究进展

EBV感染性疾病按病程可分为急性感染和慢性感染.绝大多数感染,尤其是儿童感染,是短暂、轻微且无症状的,成人的原发感染较儿童严重,病毒可能终生潜伏,但很少留有后遗症.     

慢性活动性EB病毒感染3例报告并文献复习

目的 提高对儿童慢性活动性EB病毒(CAEBV)感染的认识.方法 回顾性分析3例确诊为CAE-BV感染患儿的临床、实验室特点、治疗反应及转归.结果 3例患儿临床表现和EB病毒学检查符合CAEBV感染的临床特征,3例中2例死亡(其中1例转为恶性淋巴瘤死亡),另1例失访.结论 CAEBV感染是一种罕见的并常致命的疾病,预后较差,对于CAEBV感染患儿应早期采取积极措施,进行免疫重建,彻底消除被病毒感染或克隆增殖的淋巴细胞.     

慢性活动性EB病毒感染

慢性活动性EB病毒(EBV)感染主要表现为慢性或复发性传染性单核细胞增生症样症状,包括持续或间断发热、肝肿大、脾肿大、肝功能异常、血小板减少症、蚊虫叮咬过敏、皮疹等;表现有外周血高EBV载量和(或)异常的EBV相关抗体.该病临床少见但病死率极高,发病机制目前尚不清楚,新的诊断指南正在研究提出.传统的抗病毒治疗效果有限,新的治疗措施包括免疫抑制剂、免疫调节治疗、免疫细胞治疗等.     

儿童慢性活动性EB病毒感染临床及实验室检查特征

目的 研究慢性活动性EB 病毒感染(CAEBV)患儿的临床及外周血淋巴细胞亚群等实验室检查特征,为CAEBV 的诊治提供依据.方法 分析13 例CAEBV 患儿的临床资料,包括患儿的临床表现、病毒学检测及淋巴细胞亚群测定结果,并与15 例急性EB 病毒感染(AEBV)病例作对照研究.结果 两组患儿临床表现类似,主要为发热、肝脾肿大、淋巴结肿大等传染性单核细胞增多症(IM)样症状,区别在于CAEBV 患儿病程较长,上述症状持续或反复出现.CAEBV 组患儿外周血EBV-DNA 载量明显高于AEBV 组(P<0.05).CAEBV 组VCA-IgG 明显高于AEBV 组(P<0.05).CAEBV 组外周血白细胞计数、淋巴细胞计数、B 细胞计数、总T 细胞计数、CD4+ T 细胞计数和CD8+ T 细胞计数均低于AEBV 组(P<0.05).随访13 例CAEBV 患儿,8 例死亡,2 例好转,2 例病情仍有反复,1 例转院后失访.15 例AEBV 患儿均治愈,随访1 年无病情反复.结论 CAEBV患儿临床表现多样,早期较难与AEBV 鉴别,预后差,病死率高.外周血EBV-DNA 载量、VCA-IgG 及淋巴细胞亚群的测定对CAEBV 诊断具有一定帮助.     

儿童慢性活动性EB病毒感染

EB病毒(Epstein-Barr vims,EBV)是已知的8个人类疱疹病毒之一.可潜伏在人体B淋巴细胞中,受感染者可终身携带病毒.原发感染可无症状或出现传染性单核细胞增多症,该病具有自限性,数周后症状减轻直至消失.     

EB病毒感染与血液病

某些病毒感染可引起血液系统异常或导致血液系统疾病，如疱疹病毒中的EB病毒（EBV）、卡波西肉瘤病毒（KSHV或人类疱疹病毒-8）、巨细胞病毒（CMV）、细小病毒B19及人类免疫缺陷病毒（HIV）等。EBV、KSHV、CMV等疱疹病毒感染与宿主免疫状态密切相关。全球90％以上成人曾感染过EBV，一般均无临床症状，其体内少数B淋巴细胞中有潜伏EBV存在。     

慢性活动性EB病毒感染的研究进展

EBV感染性疾病按病程可分为急性感染和慢性感染。绝大多数感染,尤其是儿童感染,是短暂、轻微且无症状的,成人的原发感染较儿童严重,病毒可能终生潜伏,但很少留有后遗症。少数患者,急性期后发展为慢性疾病,此类EBV感染应符合以下条件:①发热、肝脾、淋巴结肿大等活动性症状反复出现或持续数月以上:②缺乏明确的基础疾病:③有特异性的抗EBV-Ab,主要是EBVCA-IgG、EA-IgG、EBNA-IgG的增高,且病变组织或外周血淋巴细胞可检测到EBV-DNA[1];④无异常或其他感染能解释上述症状,即称为慢性活动性EB病毒感染(CAEBV)[2]。CAEBV是一种…     

Rheumatic fever-mimicking carditis as a first presentation of chronic active Epstein-Barr virus infection

A 7-y-old girl presented with prolonged fever, arrhythmia and cardiomegaly during the treatment course of group A β-haemolytic streptococcal pharyngitis. The isolated rheumatogenic strain M1 suggested the diagnosis of rheumatic fever. However, serous pericardial effusion contained high levels of Epstein-Barr virus (EBV) DNA. Clonally proliferating EBV⁺ T cells were determined in the circulation. The atypical carditis without valvitis was then complicated by coronary artery dilatations. Four months after the start of prednisolone plus antiviral/bacterial therapy, EBV⁺ T-cell lymphoma developed in the thigh.
Conclusion: Atypical carditis may be a notable and life-threatening presentation of chronic active EBV infection to be differentiated from rheumatic fever.     

Chronic active Epstein-Barr virus infection in children in Japan

The patients with chronic active Epstein-Barr virus infection (CAEBV) in childhood in Japan are described. Among 39 registered cases, 20 patients were males and 19 were females. Unlike the X-linked lymphoproliferative syndrome, there was no hereditary background. The incidence of hypersensitivity to mosquito bites was high (31.3%) as a past history. Most patients exhibited hepatomegaly (92.3%), splenomegaly (87.2%) and fever (84.6%). The incidence of absent anti-EB virus nuclear antigen titres was unexpectedly low (17.1%). Lymphoreticular disorders and cardiovascular diseases were major complications. Twenty-four (61.5%) patients died 6 months to 8 years after the onset, mainly of hepatic failure (eight cases), cardiac failure (five cases), virus-associated haemophagocytic syndrome (three cases) and haematological malignancies (two cases). This study reveals that CAEBV in Japan has several clinical features and should be informative for the pathogenesis of EB virus.     

Acute thrombocytopenic purpura associated with primary Epstein-Barr virus infection

Acute idiopathic thrombocytopenic purpura (ITP) often appears to be related to the sensitization by some viral infections. However, the causative viral agents are not identified in most cases. Although the primary infection with Epstein-Barr virus (EBV) occurs during early childhood in Japan, the majority of cases are usually asymptomatic. A minority are associated with acute infectious mononucleosis (IM), which is characterized by fever, tonsillitis, lymphadenopathy, splenomegaly and liver dysfunction. In this report, three cases are described of children with EBV-induced ITP who clinically had atypical findings of IM. Their primary EBV infections were confirmed by serological test and, in addition, were verified by the enhanced expression of activation antigens (HLA-DR and CD45RO) on T cells as well as the inverted ratio of CD4⁺ to CD8⁺ subsets. These observations imply that ITP can occur as one of the host responses during primary EBV infections, irrespective of clinical manifestations. Evaluation of lymphocyte subpopulations may be useful for the assessment of primary EBV infection in ITP.     

儿童慢性活动性EB病毒感染合并UNC13D基因突变3例临床分析

目的探讨儿童慢性活动性EB病毒感染(CAEBV)合并UNC13D基因突变的临床特点,为临床诊治提供思路。方法对北京儿童医院血液肿瘤中心收治的3例CAEBV患儿进行基因突变筛查,对临床病例资料进行回顾性总结分析。结果3例患儿均存在UNC13D杂合子突变,但突变位点不同。例1和例2给予抗病毒治疗后好转出院,院外分别随访38个月和26个月,期间均出现EB病毒活动表现。例3行异基因造血干细胞移植术,随访34个月,仍无病生存。结论儿童CAEBV可能存在UNC13D基因突变,该突变可能为CAEBV发病机制以及预后差的重要原因之一。小年龄起病的发热、肝脾淋巴结肿大患儿,若EBV-DNA持续高滴度,建议尽早进行UNC13D基因突变的筛查。CAEBV患儿单纯抗病毒治疗效果差,异基因造血干细胞移植是根治性治疗手段。     

Epstein-Barr virus infection and its role in the expanding spectrum of human diseases

Recent advances of the various laboratory tests to detect Epstein-Barr virus (EBV) infection have clarified the causative role for a spectrum of EBV-associated diseases. They include lymphoproliferative disorders (LPD), which occur in immunologically compromised individuals, Hodgkin's disease (HD), chronic active EBV infection (CAEBV), virus-associated hemophagocytic syndrome (VAHS), certain forms of T cell lymphoma, and some gastric carcinomas, in addition to the classical EBV-associated diseases such as EBV genome-positive Burkitt's lymphoma (BL), undifferentiated nasopharyngeal carcinoma (NPC) and infectious mononucleosis (IM). This review intends to introduce the recent progress of studies on EBV infection mainly from the clinical points of view.     

��ͯ�ǵ���EB������Ⱦ���ٴ��ع˷���

目的探讨儿童非典型EB病毒(EBV)感染的临床特点。方法2000年3月至2005年4月,深圳市儿童医院在住院患儿中检测出EBVVCAIgM和(或)EBVDNA阳性共690例,其中传染性单核细胞增多症422例,另外268例为非典型EBV感染,对其临床特点进行回顾性分析。结果儿童非典型EBV感染以呼吸道感染最为多见(191/268,71.3%),其它为皮炎13例(4.9%)、腹泻病10例(3.7%)、血小板减少性紫癜10例(3.7%)、肾炎或肾病7例(2.6%)、肠系膜或颈淋巴结炎8例(3.0%)、结膜炎7例(2.6%),川崎病、嗜血细胞综合征、中枢神经系统感染各4例(1.5%),心肌炎、播散性脑脊髓炎各2例(0.7%),再生障碍性贫血、坏死性淋巴结炎、腮腺炎、高IgM血症、嗜酸细胞增多症各1例(0.4%),诊断不明1例(0.4%)。其中嗜血细胞综合征患儿有2例死亡。结论儿童非典型EBV感染症状多样,累及系统多,预后不一。EBVVCAIgM或EBVDNA检测有利于早期诊断,并进行合理治疗。     

Brachial neuritis following infection with Epstein-Barr virus

A case of brachial neuritis after infection with Epstein-Barr virus in a 15-year-old girl is presented. A review of the literature contrasts the presenting features in children and adults. Diagnostic tests, treatment and prognosis are discussed.