儿童噬血细胞综合征24例临床分析  

Clinical analysis of hemophagocytic syndrome in children

作　　者：边爽[1] 李程辉[1] 高晖[1] BIAN Shuang;LI Chenghui;GAO Hui(Dalian Children′s Hospital,Dalian 116000,China)

机构地区：[1]大连市儿童医院血液肿瘤科,辽宁大连116000

出　　处：《中国中西医结合儿科学》2021年第3期234-236,共3页Chinese Pediatrics of Integrated Traditional and Western Medicine

摘　　要：目的探讨噬血细胞综合征的临床特点、诊断、个体化治疗及预后。方法回顾性分析2016年9月至2019年12月我院收治的24例儿童噬血细胞综合征的临床资料。结果高热、肝脾肿大是最常见的临床表现,亦有中枢神经系统受累及急性肝功能衰竭为首发临床表现的噬血细胞综合征。全血细胞减少(29.2%)、凝血功能异常(83.3%)、甘油三酯增多(45.8%)、肝功能损害(87.5%)、乳酸脱氢酶增高(100%)、C反应蛋白升高(70.8%)、钙离子降低(25%)、钠离子降低(66.7%)、铁蛋白升高(87.5%)、自然杀伤细胞活性降低(91.7%)、可溶性白细胞介素-2受体水平升高(100%)是本组病例实验室特点。骨髓象:23例均可见成熟组织细胞和噬血细胞。结论噬血细胞综合征临床表现复杂多样,临床医生应早期认症,早期诊断,并积极寻找原发病因,及早对患者进行个体化治疗是取得良好疗效的关键。Objective To investigate the clinical features,diagnosis,individualized treatment and prognosis of hemophagocytic syndrome(HPS).Methods A retrospective study was carried out to analyze the clinical data of 24 HPS cases treated in our hospital from Sep.2015 to Nov.2018.Results The most common clinical manifestations were fever and splenomegaly.There were also central nervous involvement and acute liver failure as the first clinical manifestations of HPS.The features of laboratory data included cytopenia(29.2%),coagulation disorder(83.3%),high level of triglyceride(45.8%),hepatic function damage(87.5%),elevated lactate dehydrogenase(100%),elevated C reactive protein(70.8%),calcium ion(25%),reduced sodion(66.7%),elevated ferritin(87.5%),reduced activity of NK cells(91.75%),and elevated level of sCD 25(100%);hemophagocytosis and histiocytes could be seen in bone marrow of 23 cases.Conclusion HPS has diverse clinical manifestations;therefore,good results can be obtained by early identification,early diagnosis,and trying to find the primary cause in order to perform individualized treatment.

关 键 词：噬血细胞综合征 临床特征 个体化治疗 

分 类 号：R725.5[医药卫生—儿科]