儿童慢性活动性EB病毒感染19例临床分析  

Clinical analysis of 19 cases of chronic active Epstein - Barr virus infection in children

作　　者：刘兴楼[1] 舒赛男[1] 周华[1] 方峰[1] 

机构地区：[1]华中科技大学同济医学院附属同济医院儿科,武汉430030

出　　处：《中华实用儿科临床杂志》2016年第22期1705-1709,共5页Chinese Journal of Applied Clinical Pediatrics

摘　　要：目的分析儿童慢性活动性EB病毒感染（chronic active Epstein—Barr virus infection，CAEBV）的临床特征及预后。方法回顾性分析华中科技大学同济医学院附属同济医院儿科于2011年2月至2016年8月住院的19例CAEBV患儿的临床特点、实验室检查结果、治疗过程及预后。结果19例CAEBV患儿中，确诊时主要表现包括发热18例、肝大17例、脾大17例、淋巴结大11例及水疱样皮疹5例。血常规和肝功能结果示白细胞减少和贫血各11例，粒细胞减少8例，血小板减少2例，丙氨酸氨基转移酶升高11例，天门冬氨酸氨基转移酶升高10例。所有患儿外周血EBVDNA载量增高且病毒衣壳抗原（VCA）-IgG和核抗原（NA）-IgG阳性，早期抗原（EA）-IgG阳性8例。18例行淋巴细胞亚群分析，结果显示，自然杀伤细胞（NK细胞）明显升高7例，轻度升高1例；T淋巴细胞明显升高4例，轻度升高2例；B淋巴细胞明显升高1例；另有3例淋巴细胞亚群未见明显异常。6例行免疫基因检测者中，3例为阴性，1例有x连锁凋亡抑制蛋白基因（XIAP基因）纯合突变，2例杂合突变（其中1例突变意义尚不明确）。11例行骨髓细胞学检查，发现3例噬血细胞性淋巴组织细胞增多症（HLH），4例行淋巴结活检发现1例淋巴瘤。19例CAEBV患儿均出现并发症，其中肺炎6例，HLH、鹅口疮各3例，淋巴瘤、肠梗阻、肠穿孔、肠出血、胆囊炎、肝内胆管多发结石、自身免疫性肝炎、过敏性紫癜、肛周脓肿、营养不良及低白蛋白血症各1例。19例CAEBV患儿依据病情接受不同治疗方案，失访3例；存活8例（1例为近1年内诊断者；1例成功接受造血干细胞移植已有5年余），死亡8例，病死率为50．0％（8／16例）。结论儿童CAEBV以发热、肝脾淋巴结大、皮疹、血常规异常及转氨酶升高为主要表现；易并发肺炎和HLH等多种并发症。外周血细胞EBVDNA载量增高为�Objective To analyze the clinical characteristics and prognosis of children with chronic active Epstein - Barr virus infection （CAEBV）. Methods The clinical characteristics,laboratory test findings, treatment and prognosis of 19 cases of children with CAEBV during February 2011 to August 2016 in Department of Pediatrics ,Tongji Hospital, Tongji Medical College, Huazhong University of Science Technology were analyzed retrospectively. Results The major signs and symptoms of children with CAEBV were fever （18 cases） , hepatomegaly （17 cases） , splenomegaly （17 cases）,lymphadenopathy （11 cases） and blister -like rash （5 cases ）. Blood routine and liver function results showed leukopenia （ 11 cases） and anemia （ 11 cases ） , neutropenia （ 8 cases ） , thrombocytopenia （ 2 cases ） , elevated alanine aminotransferase （11 cases）, elevated aspartate aminotransferase （10 cases）. EBV DNA load increased, viral capsid antigen（VCA） -IgG and nuclear antigen （NA） - IgG were positive in peripheral blood of all cases, and early antigen（EA） - IgG was positive in 8 cases. The results of lymphocyte subsets in 18 cases showed that the proportion of natural killer cell（ NK cell） significantly increased in 7 cases, and 1 case was slightly elevated. The proportion of T cells significantly increased in 4 cases, and 2 cases were slightly elevated. The proportion of B cells significantly increased in 1 case ; but the results of lymphocyte subsets had no obvious abnormalities in other 3 cases. Genetic testing of 6 cases showed that 3 cases were negative, 1 case with a homozygous mutation in the X - linked inhibitor of apoptosis protein gene（XIAP gene） ,and 2 cases had heterozygous mutation （the meaning of mutation was unclear in 1 case）. Three out of 1l cases had hemophagocytic syndrome by bone marrow examination, and 1 case out of 4 cases had lymphoma by lymph node biopsy. All of 19 cases of CAEBV had complications, including 6 cases of pneumonia, 3 cases of hemo- phago

关 键 词：慢性活动性EB病毒感染 临床特征 诊断 治疗 预后 儿童 

分 类 号：R725.1[医药卫生—儿科]