小儿感染相关性噬血细胞综合征31例检验结果分析

目的分析小儿感染相关性噬血细胞综合征(IAHS)的临床及实验室特点,探讨IAHS早期诊断指标。方法分析2004年5月至2008年6月收治的31例IAHS的临床及实验室资料。结果EB病毒感染者18例(58.1%)、CMV感染4例(12.9%)、细菌感染4例(12.9%)、真菌感染3例(9.7%)、立克次氏体感染1例(3.2%)和HBV感染1例(3.2%);本组病例以持续高热(100%)、肝肿大(74.2%)、脾肿大(71%)为主要表现;外周血细胞白细胞计数减少(93.5%),血小板减少(87.1%),血清甘油三酯高(90.3%),铁蛋白高(96.8%),肝功能异常(67.7%),凝血异常(64.5%),100%骨髓找到噬血细胞;早期IAHS肝功能和凝血功能改变不明显。结论典型IAHSI临床以持续高热,肝、脾肿大为主要表现,实验室检查以白细胞计数降低、血小板低、肝功能及凝血功能异常、甘油三酯及铁蛋白增高,骨髓找到噬血细胞为主要特点。噬血细胞的形态特征、肝功能及凝血功能异常程度有助于分析IAHS早期诊断。

Clinical analysis of 31 cases of pediatric Infection associated hcmophagocytic syndrome

Objective To explore the clinical characteristic and early experimental indexes changes on pediatdc infection associated hemophagocyfic syndrome(IAHS). Methods The clinical and laboratory data of 31 cases of IAHS were retrospectively analyzed. Results In all the 31 cases of IAHS,18 cases were infected with Epstein-Bart virus(58.1%),4 cases CMV(12.9%),4 cases bacterial(12.9%),3 cases ftmg(9.7%),I case HBV and I case Rickettsia(3.2%). All patients had fever and were found hemophagocytic histiocytes in marrow slides,the main manifestutions were hepatomegaly(74.2%),splenomegalin(71%),hypertriglyceridemia(90.3%), hypefferritinmia(96.8%),hepatic lesion(67.7%),coagulative dysfunction(64.5%) and the number of WBC(93.5%) and PLT(87.1%) were reduced in peripheral blood. Conclusion The main mainfestutions of IAHS were continuing hyperpyrexia,hepatomegaly and splenomegalin,and the laboratory data showed pancytopenia,hepatic lesion,and the hemophagocytic histiocytes were observed.The charactical of hemophagocytic histiocytes and the degree of hepatic lesion and coagulative dysfunction were helpful in diagnose earl),IAHS.