35例噬血细胞综合征临床特征与预后因素分析

目的总结噬血细胞综合征患儿的临床特征,探讨其预后及死亡危险因素。方法回顾性分析2003年1月~2009年12月我院收治的35例噬血细胞综合征患儿的临床特点、实验室检查及治疗。并采用SPSS10.0软件进行多因素Logistic回归分析,探讨患儿死亡的危险因素。结果 35例患儿中20例与感染相关（57.1%）,尤其是EBV感染诱发。其他原因4例（11.4%）,原因不明11例（31.4%）。临床表现发热34例（97.1%）,肝肿大31例（88.6%）、脾脏肿大23例（65.7%）,淋巴结肿大14例（40.0%）。实验室检查表现为不同程度的血细胞减少,其中贫血31例（88.6%）,血小板降低27例（77.1%）,白细胞降低18例（51.4%）。肝功能异常（100%）,高三酰甘油血症13例（37.1%）,低纤维蛋白原血症14例（40.0%）,血清铁蛋白增高15例（42.8%）,骨髓涂片找到噬血细胞33例（94.3%）。预后危险因素分析显示Hb〈30g/L是预后不良的危险因素,病死率高。结论噬血细胞综合征病因复杂,临床表现多样,病死率较高,早期诊断、早期化疗可提高患儿的生存率。

Clinical characteristics of 35 children with hemophagocytic syndrome and prognosis analysis

Objective To investigate the clinical features,diagnosis and treatment of childhood hemophagocytic syndrome（HPS） and possible factors affecting prognosis.Methods Thirty-five children with HPS from Jan.2003 to Dec.2009 were retrospectively analyzed,their clinical features,laboratory data,treatments and prognosis factors were reviewed.Logistic regression analysis was performed to determine possible factors affecting prognosis.Results Among 35 children with HPS,20（57.1%） were related with infection,of which EB virus-associated HPS was most common.4（11.4%）were other causes,while the etiology of another 11 cases（31.4%） was unknown.Main clinical features： fever（97.1%）,hepatomegaly（88.6%）,splenomegaly（65.7%）,enlarged superficial lymph nodes（40.0%）.Laboratory tests showed anaemia（88.6%）,thrombocytopenia（77.1%）,decreased count in white blood cell（51.4%）,liver dysfunction（100%）,hypertriglyceridema（37.1%） and（or） decreased fibrinogen（40%）,increased ferritin（42.8%）,hemophagocytosis in bone marrow（94.3%）.Logistic regression analysis indicated that Hb30g/L was independent adverse prognostic factors,with high mortality.Conclusion Childhood HPS presented complicated causes,diverse clinical characteristics and high mortality.Recognize,diagnose and treat it with HLH-2004 project as early as possible are the key to improve prognosis.