EB病毒相关性噬血细胞综合征的临床特征及外周血淋巴细胞亚群的变化

Clinical features and changes of peripheral blood lymphocyte subsets in children with EBV-associated hemophagocytic syndrome

目的 分析EB病毒相关性噬血细胞综合征（EBV-AHS）患儿的临床特点,了解其外周血淋巴细胞亚群的变化.方法 20例EBV-AHS患儿,均符合噬血细胞综合征的诊断标准,并有EBV感染的证据,对其临床特点进行总结分析,并采用流式细胞术检测外周血淋巴细胞亚群CD4＋、CD8＋、CD19＋、CD16＋CD56＋、CD4＋/CD8＋的水平.选择20例健康儿童作为对照组.结果 在EBV-AHS的诊断标准中,以发热、血常规2系以上降低、乳酸脱氢酶升高（＞1 000 U/L）、高密度脂蛋白降低及铁蛋白增高（＞1 500 g/L）5项指标异常发生率最高,均为100%.与对照组相比,EBV-AHS患儿外周血CD8＋T及CD19＋B细胞比例增高（分别为53.70% ±10.6% vs 27.05% ±8.22%,24.95% ±5.34% vs11.85% ±4.53%）,而CD4＋T及CD16＋CD56＋NK细胞比例下降（分别为23.60% ±6.42% vs 45.20% ±5.74%,5.55% ±2.87% vs 14.70% ±4.16%）,CD4＋/CD8＋比值降低（0.46±0.16 vs 1.84±0.68）,差异均有显著性（P＜0.01）.结论 EBV-AHS患儿细胞及体液免疫功能发生紊乱,导致了疾病的发生发展.

Objective To summarize clinical features and changes of peripheral blood lymphocyte subsets in children with EBV-associated hemophagocytic syndrome. Methods Twenty children diagnosed with EBV-AHS were characterized by hemophagocytic syndrome and had the evidence of EBV infection.Clinical characteristics were analyzed and proportions of lymphocyte subsets of CD4＋ ,CD8＋, CD19＋,CD16＋CD56＋ and CD4＋/CD8＋ in peripheral venous blood were determined by flow cytometry. Twenty healthy children matched in age and gender with these patients were selected as control. Results In all the diagnostic items of EBV-AHS, fever, decline of cells over two lineages in blood routine, enhanced lactate dehydrogenase （ 〉1 000 U/L） and ferritin （ 〉 1 500 g/L）,decreased high-density lipoprotein occurred in all the patients with an incidence of 100%. The percentages of CD8 ＋ T and CD19 ＋ B in children with EBV-AHS were higher than those in control group（53.70% ± 10.6% vs 27.05% ±8.22%,24.95% ±5.34% vs 11.85 % ± 4. 53 %, respectively）, while levels of CD4 ＋ T and CD16 ＋ CD56 ＋ NK cell were lower compared with control group （23.60% ±6. 42% vs 45.20% ±5.74% ,5.55% ±2. 87% vs 14. 70% ±4. 16% ,respectively ,P 〈 0.01 ）. Conclusion Disorder of cellular and humoral immunity happens in patients with EBV-AHS, which could be implicated in the pathogenesis and progression of EBV-AHS.