EB病毒相关性噬血细胞综合征患儿的临床观察及实验室检查结果分析

The efficacy and analysis of laboratory results in pediatric patients with EB virus associated hemophagocytic syndrome

目的总结儿童噬血细胞综合征患者的临床和实验室特点。方法采用回顾性分析法总结我院2012-2013年确诊的6例EB病毒相关性噬血细胞综合征患儿的临床表现及实验室诊断指标结果,并对相关数椐进行分析。结果 6例患儿临床主要表现为反复发烧、肝脾淋巴结肿大,继而出现黄疸及呼吸道感染等症状,实验室检查主要表现为血细胞进行性减少,乳酸脱氢酶(LDH)等酶类升高,凝血功能检测指标异常,EB病毒DNA拷贝数均增高。骨髓细胞学检查均见吞噬血细胞现象。结论反复发烧、肝脾淋巴结肿大、呼吸道感染等临床特征结合相应实验室检查结果,有助于噬血细胞综合征的诊断,从而进行早期治疗。

Objective To summarize and analyze the clinical and laboratory characteristics of pediatric patients with hemophagocytic syndrome in order to improve early diagnosis of this disease in children. Methods Between 2012 and 2013, 6 hospitalized children who were diagnosed as EB virus associated hemophagocytic syndrome in our hospital were enrolled into this study and their clinical manifestations and laboratory examination results were analyzed retrospec-tively. Result The main clinical manifestations of the 6 cases were periodic fever, liver, spleen and lymph nodes enlarge-ment, followed by jaundice and lung involvement and other symptoms. Laboratory tests showed that the peripheral blood cells were progressively decreased, LDH level increased and phagocytic phenomena were seen in bone marrow cytology, with abnormal liver function and coagulation dysfunction. EB virus DNA copy number was positive. Conclusion The clinical features of repeated fever, liver, spleen and lymph node enlargement, respiratory tract infection and the character-istic laboratory test results could be hints for early diagnosis of the hemophagocytic syndrome. Early treatment may improve the prognosis.