小儿噬血细胞综合征的发病机制及预后危险因素的分析
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科技部973项目(2011CB504000);国家自然基金面上项目(81070656,81171589);江苏省特聘医学专家科研资助专项资助


The pathogenesis and prognosis of infantile eats blood syndrome
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    摘要:

    目的:探讨儿童噬血细胞综合征(white blood cell syndrome,HPS)的临床特点-诊断标准-治疗过程以及预后的相关危险因素。方法:回顾分析2009—2013年南京市儿童医院血液肿瘤科收治的72例儿童HPS的临床表现-辅助检查-治疗及转归,采用Logistic方法分析患儿的预后危险因素。结果:72例中42例(58.3%)是感染相关性HPS,其中EB病毒感染相关性HPS最多;其余的30例中4例(5.56%)是非感染相关HPS,26例(36.1%)病因不明。临床表现为发热(91.7%)-肝脏肿大(83.3%)-脾脏肿大(66.7%)。外周血血常规三系检查结果为两系减低(72.2%),三系低下(27.8%),血红蛋白浓度减低(91.7%),血小板数量减少(77.8%);血生化检查血清甘油三酯升高(38.9%);凝血常规检查功能障碍(41.7%);血清铁蛋白升高(55.6%);骨髓细胞形态学检查大部分找到了噬血细胞。72例中死亡10例(13.8%),好转或者基本痊愈24例(33.3%),出院后失访14例(19.4%)。年龄<3岁-乳酸脱氢酶(LDH)>2 500 U/L是不利的危险因素。结论:年龄小和LDH水平增高是儿童HPS预后不良的危险因素,应该及早诊断治疗,降低病死率。

    Abstract:

    Objective:To investigate the clinical characteristics of children’s white blood cells syndrome(HPS),diagnostic criteria,treatment process and related risk factors of prognosis. Methods:A retrospective analysis was conducted in 72 cases of children’s blood oncology of HPS treated in of 2009—2013 in Nanjing Children’s Hospital. Clinical manifestation,auxiliary examination,treatment and outcome,adopting Logistic methods were used to analyze the prognosis of children with risk factors. Results:In 72 cases,42 cases (58.3%) was HPS infection correlation,among which correlated with EB virus infection was the most conmon type. In the rest 30 cases,4 cases(5.56%) were related HPS infection,and 26 cases(36.1%)were unknown etiology. Clinical manifestatios included fever (91.7%),liver enlargement (83.3%),and spleen enlargement (66.7%). Peripheral blood routine test showed that the two of three lines decrease accounting to 72.2%,three lines decreased accounting to 27.8%,hemoglobin concentration reduction was 91.7%,the decrease of platelets was 77.8%. Elevated serum triglyceride was 38.9%. Coagulation routine inspection dysfunction was 41.7%. Elevated serum ferritin was 55.6%. Bone marrow cell morphology most found the white blood cells. Seventy-two cases of death in 10 cases (13.8%),improvement or basic recovery 24 cases (33.3%),14 cases (19.4%) lost to follow-up after discharge. Age < 3 years old, LDH > 2 500 U/L were the risk factos for adverse. Conclusions:Age and LDH leels are children that eats blood syndrome risk factors of poor prognosis,and should be treated early diagnosis and reduce the case fatality rate.

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张雯雯,陈红兵,朱 君,常 林,韩晶晶,苏东明.小儿噬血细胞综合征的发病机制及预后危险因素的分析[J].南京医科大学学报(自然科学版),2015,(8):1118-1122

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  • 收稿日期:2015-01-17
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  • 在线发布日期: 2015-08-04
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