儿童EB病毒相关淋巴组织增殖性疾病临床分析

目的:分析儿童EB病毒(Epstein-Barr virus,EBV)相关淋巴组织增殖性疾病的临床特点,提高对该病的认识。方法:对浙江大学医学院附属第一医院2014年1月至2018年12月收治的确诊EBV相关淋巴组织增殖性疾病患儿的临床资料以及治疗进行回顾性总结。根据疾病病程和严重程度分为轻症组和重症组。采用 ...     

儿童EB病毒感染相关淋巴增生性疾病的诊治进展

EB病毒是人类于1964年发现的第一个致癌病毒。EB病毒感染在儿科较为常见,多无临床症状。当患儿免疫功能降低或缺乏时,EB病毒可能会引起传染性单核细胞增多症、慢性活动性EB病毒感染、噬血细胞性淋巴组织细胞增生症、移植后淋巴增生性疾病、X连锁淋巴组织增生综合征等一系列良性或恶性淋巴增殖性疾病。这些疾病大部分发病机制复杂、...     

EB病毒相关的淋巴增生性疾病的诊断与治疗

EB病毒(EBV)感染与多种临床疾病密切相关,特别是多种淋巴增生性疾病,临床表现多样,慢性病程或经过凶险,大部分预后不良,为临床诊断和治疗的难点。现介绍部分相关疾病的临床诊断标准和治疗。     

EB病毒相关恶性肿瘤研究进展

EB病毒（Epstein-Barrvirus,EBV）属疱疹病毒科γ亚科中惟一能引起人类感染的淋巴滤泡病毒,近50年来的研究证明EBV与淋巴瘤、白血病、移植后淋巴增生性疾病、鼻咽癌、胃癌等多种人类肿瘤发生有关。由于EBV对人类的普遍易感性,近年有关EBV的免疫学特性、逃避机体免疫应答的机制、EBV感染相关疾病的致瘤机制以及治疗方法等研究有了很大进展,本文就相关问题进行阐述。     

EB病毒感染及其相关性疾病

EB病毒(EBV)感染是儿科较为常见的病毒感染性疾病,不同的免疫状态有不同的临床表现。机体感染EBV后,可以产生传染性单核细胞增多症、EBV相关性噬血细胞综合征、慢性活动性EBV感染、X连锁淋巴组织增生性疾病及鼻咽癌、淋巴瘤等。免疫缺陷患者感染EBV后容易发生淋巴组织增殖性疾病。传染性单核细胞增多症是EBV感染最常见的临床表现形式,常合并一系列的并发症。     

EB病毒感染及其相关性疾病

EB病毒（EBV）感染是儿科较为常见的病毒感染性疾病，不同的免疫状态有不同的临床表现。机体感染EBV后，可以产生传染性单核细胞增多症、EBV相关性噬血细胞综合征、慢性活动性EBV感染、X连锁淋巴组织增生性疾病及鼻咽癌、淋巴瘤等。免疫缺陷患者感染EBV后容易发生淋巴组织增殖性疾病。传染性单核细胞增多症是EBV感染最常见的临床表现形式，常合并一系列的并发症。     

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。     

Primary Immunodeficiency Diseases and Epstein-Barr Virus-induced Lymphoproliferative Disorders

Increased incidence of malignant disorders is noted in patients with both primary and acquired immunodeficiency diseases. The pathogenetic mechanism(s) for these disorders remain unclear. Defective immunosurveillance of these patients, however, is mainly postulated to be responsible for the increased risk of these malignant disorders. Of the malignant disorders, Epstein-Barr virus (EBV)-induced lymphoproliferative disorders (LPD) have been increasingly reported, possibly due to improved therapeutic management techniques such as bone marrow transplantation, which results in prolonged survival periods for the primary immunodeficiency; the dramatic development of immunosuppressive treatments for transplant recipients; and the growing numbers of acquired immunodeficiency syndrome (AIDS) patients.
This review focuses on the primary immunodeficiency diseases and EBV-induced LPD, and discusses pathogenetic mechanism(s) for the increased incidence of these malignant disorders.     

Epstein-Barr Virus Polymerase Chain Reaction and Serology in Pediatric Post-Transplant Lymphoproliferative Disorder: Three-Year Experience

To assess whether the semiquantitative peripheral blood Epstein-Barr virus (EBV) polymerase chain reaction (PCR) test correlates with post-transplant lymphoproliferative disorder (LPD), we compiled the results of the test done over a 3-year period ending July 1997. Six hundred seventy-six tests were done on 185 patients. Four hundred-thirty tests (63%) were negative, 167 (25%) were weak positive, 67 (10%) were moderate positive, and 12 (2%) were strong positive. Twelve of the patients developed a lymphoproliferative disorder (LPD) during this time. The EBV PCR tests proximate to the diagnosis of LPD in the 12 patients with EBV-positive LPD were 6 strong positive, 5 moderate positive, 1 weak positive. No patient with LPD had a negative result at diagnosis. Stated another way, 6/12 (50%) of strong-positive PCR tests, 5/67 (7%) moderate-positive tests, and 1/167 (.6%) of weak-positive tests correlated with LPD. Serologic evaluation for EBV done on 7 patients at the time of LPD showed low serologic responses in 5 of the 7 patients. The EBV PCR temporally associated with the serology indicated moderate to large viral burdens. In each patient evaluated serially, the EBV PCR test rose before the diagnosis of LPD and fell with treatment for the disorder. In conclusion, the EBV PCR test may be used as an adjunct to the diagnosis of patients with LPD and may be used to monitor response to therapy for the disorder. Received August 26, 1997; accepted January 13, 1998.     

Epstein-Barr Virus–Associated Lymphoproliferative Disorder Presenting as Apparently Isolated Gastrointestinal Lesions in Childhood

We present an unusual case of post-transplant lymphoproliferative disorder (PTLD) presenting as apparently isolated gastrointestinal lesions in a pediatric renal transplant recipient. The multiple bowel lesions were related to Epstein-Barr virus and demonstrated the appearance of a monomorphic PTLD that was morphologically indistinguishable from diffuse large B-cell lymphoma. The patient responded to therapy with targeted anti-CD20 immunotherapy. PTLD may manifest as apparently isolated gastrointestinal tract lesions in childhood.     

Ataxia Telangiectasia with Persistent Epstein-Barr Virus Infection and Malignant Lymphome

Ataxia telangiectasia is agenetically determined immunodeficiency with predisposition to malignancy. Herein we report a case of ataxia telangiectasis in a child who had showed high antibody titers to viral capsid antigen (VCA), presence of antibodies to Epstein-Barr virus (EBV) induced early antigen (EA), low titers of antibodies to EBV associated nuclear antigen (EBNA), and a high incidence of EBNA-positive cells in the peripheral blood without symptoms of infectious mononucleosis before succumbing to malignant lymphoma five years later. We hypothesize the association betwen some cytogenetic defects and persistent EBV infection on the development of the malignant lymphoma in this patient.