儿童慢性活动性EB病毒感染的相关血液疾病特征  

Characteristics of Chronic Active Epstein-Barr Virus Infection-associated Hematological Disorders in Children

作　　者：刘英[1] 唐锁勤[1] 刘立真[1] 杨光[1] 冯晨[1] 雷琦[1] 

机构地区：[1]解放军总医院小儿内科,北京100853

出　　处：《中国实验血液学杂志》2008年第3期574-578,共5页Journal of Experimental Hematology

摘　　要：本研究通过4个病例分析儿童慢性活动性EB病毒(CAEBV)感染相关的血液学病征。总结了临床特点,应用显微镜观察骨髓涂片的细胞形态,流式细胞仪分析淋巴细胞亚群,免疫组织化学检测肝穿刺组织,酶联免疫吸附试验(ELISA)检测血浆EBV抗体,实时定量PCR检测血浆EBV-DNA,原位杂交检测外周血单个核细胞(PBMNCs)EBV编码小RNA-1(EBER-1)。结果显示:4例患儿有持续或反复的发热、肝脾肿大、肝功能异常、贫血、血小板减少症、全身炎症反应;骨髓象表现增生减低、成熟障碍、病态造血和噬血细胞增多;4例患者均有CD8+T淋巴细胞增多,其中1例进展为T细胞淋巴瘤;2例EBV-VCA-IgG滴度≥1∶5120,1例血浆EBV-DNA拷贝数为3·26×103/ml,1例PBMNC的EBER1阳性率为1·7%;最后,4例患者均被诊断为CAEBV。结论:免疫相关性血细胞减少、巨噬细胞活化综合征和淋巴细胞增殖性疾病等是本研究中4例CAEBV患儿的血液学病征。The aim of this study was to analyze characteristics of chronic active Epstein-Barr virus （CAEBV） infection associated hematological disorders in children. Clinical characteristics were summaried; the morphology of hematopoietic cells in bone warrow was observed by microscopy; the lymphocyte subpopulations were analyzed by flow cytometry; the immunophenotype of liver biopsies was assayed by immunohistochemistry; EBV-related antibodies were measured by ELISA; serum EBV-DNA loads were detected by real-time quantitative PCR; EBV-encoded small RNA 1-positive cells in peripheral blood mononuclear cells were identified by in situ hybridization. The results indicated that the clinical manifestations in patients included persistent or recurrent fever, hepatoslSlenomegaly, liver dysfunction, anemia, thrombocytopenia, systemic inflammatory reaction. Bone marrow presented as hypocellularity, dysmaturation, myelodysplasia and hemophagocytosis. CD8^＋ cell high counts were demonstrated in all 4 patients, one of them developed into a T cell lymphoma. Serum EBV-DNA load was 3.26 × 10^3 copies/ml in one patient, EBER1^＋ cells were detected at a frequency of 1.7% in PBMNCs from another patient; the titers of IgG to EBV-VCA were ≥ 1 ： 5 120 in the rest 2 patients. All 4 patients described above were diagnosed as CAEBV infection. In conclusion, the immune-related cytopenia, macrophage activation syndrome and lymphoproliferative disorders are characteristics of CAEBV infection associated hematological disorders in these 4 children patients.

关 键 词：EB病毒感染 慢性活动性EB病毒感染 免疫相关性血细胞减少 巨噬细胞活化综合征 淋巴细胞增殖性疾病 

分 类 号：R552[医药卫生—血液循环系统疾病] R733[医药卫生—内科学]