EB 病毒相关性噬血细胞性淋巴组织细胞增生症：中国大陆 78 例儿科病例的回顾性研究。

Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis: a retrospective study of 78 pediatric cases in mainland of China.

机构信息

Department of Infection, Beijing Children's Hospital, Capital Medical University, Beijing 100045, China.

出版信息

Chin Med J (Engl). 2010 Jun;123(11):1426-30.

PMID:20819601

Abstract

BACKGROUND

The clinical characteristics of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) are largely unreported in the pediatric patients in mainland of China. The main aim of this study was to recognize the clinical features of EBV-HLH in children and to explore its prognosis and risk factors.

METHODS

A retrospective study was performed on 78 pediatric patients with EBV-HLH who were admitted to Beijing Children's Hospital between 2003 and 2008. All patients' medical records were reviewed and analyzed. For each patient, demographic, clinical, laboratory and outcome information was collected. Statistical analysis was conducted via multivariate and univariate analysis.

RESULTS

The age of onset peaked between 1 - 2 years and boys were more likely developed EBV-HLH. EBV-HLH occurred mainly in the serological pattern with EBV nuclear antigen (EBNA) positive (70.5%). The overall fatality of the disease was 56.7%. Twelve of the 39 fatalities (30.8%) died rapidly within 2 months after diagnosis. Multivariate analysis revealed that not receiving chemotherapy (P = 0.002), > or = 4 weeks of illness prior to diagnosis (P = 0.004), and albumin levels < 20 g/L (P = 0.045) significantly predicted an increased fatality risk.

CONCLUSIONS

EBV-HLH is a severe disease with a high fatality rate that occurs mainly in the serological pattern with EBNA positive. Early initiation of chemotherapy and timely diagnosis significantly improves survival rate. Practical strategies should focus on reducing the likelihood of early death.

摘要

背景

中国大陆儿童中 EB 病毒相关性噬血细胞性淋巴组织细胞增生症（EBV-HLH）的临床特征尚未得到充分报道。本研究的主要目的是认识中国儿童 EBV-HLH 的临床特点，探讨其预后及危险因素。

方法

对 2003 年至 2008 年北京儿童医院收治的 78 例 EBV-HLH 患儿进行回顾性研究。对所有患儿的病历进行分析，记录其人口统计学、临床、实验室及转归资料。采用多因素及单因素分析。

结果

发病年龄高峰为 1 至 2 岁，男性多见。EBV-HLH 主要发生在 EBV 核抗原（EBNA）阳性的血清学模式（70.5%）。该病总病死率为 56.7%，39 例死亡病例中 12 例（30.8%）在诊断后 2 个月内迅速死亡。多因素分析显示未接受化疗（P = 0.002）、起病至诊断时间 > 4 周（P = 0.004）及白蛋白 < 20 g/L（P = 0.045）与病死率增加显著相关。