| 儿童合并骨髓噬血现象53例临床分析 |
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| 引用本文： | 杨颖哲,汤静燕,薛惠良,陈静,潘慈,顾龙君,宋得莲.儿童合并骨髓噬血现象53例临床分析[J].临床儿科杂志,2009,27(8). |
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| 作者姓名： | 杨颖哲 汤静燕 薛惠良 陈静 潘慈 顾龙君 宋得莲 |
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| 作者单位： | 上海交通大学医学院附属上海儿童医学中心血液肿瘤科,上海,200127 |
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| 摘 要： | 目的 探讨骨髓中出现噬血现象患儿的临床特点、诊断、治疗及其与预后之间的关系.方法 回顾性分析1998年-2008年间骨髓发现噬血现象的53例住院患儿的临床特征、实验室检查、原发病诊断、治疗方法及预后.结果 共53例纳入统计,其中男35例,女18例;中位年龄3岁(2个月～16岁).出院诊断噬血细胞综合征(HLH)20例,肿瘤9例,再生障碍性贫血1例,幼年性特发性关节炎2例,特发性血小板减少性紫癜2例,郎格罕斯组织细胞增生症2例,尼曼匹克病1例,黄色肉芽肿1例,传染性单核细胞增多症1例,原发败血症1例,病情恶化自动出院或死亡时未明确诊断13例.根据国际组织细胞协会HLH诊断指南-2004标准重新评估,符合HLH诊断标准22例.是否存在脾肿大、外周血≥二系细胞减少、高三酰甘油血症或低纤维蛋白血症及骨髓噬血现象对HLH诊断意义较大.激素治疗组患儿预后较好,是否符合HLH诊断标准与预后相关性不大.结论 骨髓噬血现象并非某个疾病的特异性表现,噬血细胞综合征的诊断也缺乏特异性指标,在治疗的选择上不应仅仅依靠诊断标准,而应综合考虑每个患儿的具体情况.早期开始激素治疗对预后有一定帮助.完善分子学诊断刻不容缓.
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| 关 键 词： | 儿童 噬血现象 预后 治疗 |
Clinical study on fifty-three children with hemophagocytic phenomenon |
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| FANG Ying-zhe,TANG Jing-yan,XUE Hui-liang,CHEN Jing,PAN Ci,GU Long-jun,SONG De-lian.Clinical study on fifty-three children with hemophagocytic phenomenon[J].The Journal of Clinical Pediatrics,2009,27(8). |
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| Authors: | FANG Ying-zhe TANG Jing-yan XUE Hui-liang CHEN Jing PAN Ci GU Long-jun SONG De-lian |
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| Institution: | Department of Hematology/Oncology;Shanghai Children's Medical Center;Shanghai Jiaotong University School of Medicine;Shanghai 200127;China |
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| Abstract: | Objective To discuss the relationship between clinical characteristics,diagnosis,therapy and outcome for children with hemophagocytic phenomenon in bone marrow.Methods A retrospective analysis of the clinical signs,laboratory data,diagnosis,therapy and outcome of patients with hemophagocytic phenomenon in bone marrow from January 1998 to December 2008 was performed.Results A total of 53 cases,35 males and 18 females,were investigated.Mean age was 3 years old(ranged from 2 months to 16 years old).At the time... |
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| Keywords: | children hemophagocytic phenomenon outcome therapy |
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