四川医学2011，Vol.32Issue(5)：695-698,4.
35例噬血细胞综合征临床特征与预后因素分析
Clinical characteristics of 35 children with hemophagocytic syndrome and prognosis analysis
李蕙 1周敏 1徐鸣 1李丹 1蒋永梅 1史宏1
作者信息
- 1. 成都市儿童医院,四川,成都,610017
- 折叠
摘要
Abstract
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 Hb ＜ 30g/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.关键词
噬血细胞性综合征/儿童/临床特点/危险因素Key words
hemophagocytic syndrome/ children/ clinical characteristics/ prognostic factors分类
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李蕙,周敏,徐鸣,李丹,蒋永梅,史宏..35例噬血细胞综合征临床特征与预后因素分析[J].四川医学,2011,32(5):695-698,4.
