Objective To investigate the clinical characteristics of childhood hemophagocytic syndrome( HPS) and possible death risk factor. Methods A retrospective study was conducted to analyze the cause, clinical characteristics, laboratory findings and outcomes of 54 patients with HPS. Results Fever was the most common symptom ( 100% ) in the 54 HPS patients. They also had other features including splenomegaly, hepatomegaly, lymphadenopathy, jaundice, dropsy of multiple serous cavity, oedema, skin purpura, respiratory symptoms, alimentary canal haemorrhage, skin rash, urinary haemorrhage and central nervous system symptoms. Liver dysfunction which was manifested by elevated liver enzymes ( ALT, AST and LDH) was the most significant feature in laboratory examination. The other features of laboratory examination were hemophagocytosis in bone marrow, decrease of 2 lineages cell in the peripheral blood, hypertriglyceridemia, hyperferritin and hypofibrinogenemia. The pathogenic study showed that the underlying disease of infection ( IAHS) was the most common cause of this disease ( 72% ) , in which EBV-AHS was predominant( 86% ) .In the 54 cases, 25 cases were died( the mortality was 46% ) , 19 of them were IAHS( 17 cases were EBV-AHS, 2 cases were septicemia) , 2 cases were malignant tumors, 4 cases were undetermined. The univariate analysis showed that variables significantly associated with death in HPS were age, EBV-infection, thrombocytopenia, elevated ALT and LDH. The Logistic regression showed that variable significantly associated with death were EBV-infected, thrombocytopenia and the elevated ALT. Conclusions EBV infection is the most common cause of HPS. EBV-infection, thrombocytopenia and the elevated level of ALT are death-associated risk factors. Treatment with HLH-2004 protocol may improve the survival of HPS.%目的 分析儿童噬血细胞综合征(HPS)的临床特征及影响预后的危险因素,以提高本病的早期诊断率,减少误诊.方法 回顾性分析54例儿童HPS的病因、临床特征、实验室检查结果及转归.结果 54例患儿均以发热为首发症状(100%),其余临床表现按发生率多少依次为脾脏肿大、肝脏肿大、淋巴结肿大、黄疸、多浆膜腔积液、水肿、呼吸系统症状、消化道出血、皮疹、泌尿系出血、中枢神经系统症状.实验室检查以肝功能异常最为突出(100%),以酶学(ALT、LDH、AST)改变为主(100%),其后依次为骨髓见噬血细胞、外周血至少两系细胞减少、高甘油三酯血症、血清铁蛋白升高、低纤维蛋白原血症、NK细胞活性降低、肾功能异常.病因分析显示,感染相关性(IAHS)占72%,以EB病毒相关性噬血细胞综合征(EBV-AHS)最多,占IAHS的82%.54例HPS患儿中,自动出院19例(随访中16例死亡),接受治疗35例,9例(26%)死亡,21例治愈或有效(60%),5例正在治疗中.死亡25例中,IAHS19例(EBV感染17例,败血症2例),恶性肿瘤2例,原因不明者4例.单因素分析显示发病年龄、EBV感染、血小板计数降低、LDH和ALT水平升高与患儿死亡有关.将以上因素进行Logistic多因素分析,显示EBV感染、血小板降低、ALT升高是噬血细胞综合征患儿死亡的独立危险因素.结论 HPS由多种病因所致,临床表现多样,预后凶险.病因分析,EBV-AHS占首位.EBV感染、PLT计数、ALT水平是影响HPS预后的独立危险因素,采用HLH-04方案治疗有助于提高HPS患儿的生存率.
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