噬血细胞综合征(HPS)的早期鉴别诊断和实验室结果分析  

Analysis of the Etiology and Clinical Features of Bone Marrow in Children with Hemophagocytic Phenomenon

作　　者：王玲[1] 尹春燕[1] 肖延风[1] 郭漫[1] 毛华[1] 李红艳[1] 

机构地区：[1]西安交通大学第二附属医院儿科

出　　处：《现代检验医学杂志》2016年第4期107-109,共3页Journal of Modern Laboratory Medicine

摘　　要：目的 分析儿童骨髓噬血细胞现象的病因和临床特点,为噬血细胞综合征（HPS）的早期诊断和鉴别诊断提供依据。方法 收集73例骨髓片存在噬血现象患儿的临床资料,根据是否达到噬血细胞综合征的诊断标准进一步分为HPS组和非HPS组,分析两组患儿的病因、诊断、临床特点、血常规、生化、血脂、凝血、铁蛋白及NK细胞,并进行统计学分析。结果 ①所有HPS患儿起病时均有发热,占100%; 28例非HPS患儿存在发热,占所有非HPS患儿的71.79%; 两组患儿发热率比较,差异具有统计学意义（χ^2=4.62,P〈0.05）。②HPS组患儿二系减少,高三酰甘油、低纤维蛋白原、高铁蛋白和低NK细胞发生率显著高于非HPS组,差异具有统计学意义（χ^2=3.51-6.36,P〈0.05）。③HPS组白细胞总数、中性粒细胞、红细胞、血红蛋白和血小板均显著低于非HPS组,差异具有统计学意义（t值=3.01-5.62,P〈0.05）。④在病因分析上,HPS和非HPS均以感染性疾病居多,非HPS还可见川崎病、全身性幼年类风湿关节炎等疾病。结论 儿童骨髓中出现噬血现象,并非仅见于HPS,需根据临床症状和检验结果综合评价。Objective To analyze the etiology and clinical features of bone marrow in children with hemophagocytic phenome- non and provide a theoretical basis for early diagnosis and differential diagnosis of hemophagocytic syndrome （HPS）. Methods The clinical data of 73 cases whose bone marrow smears existed hemophagocytie phenomenon were collected in two years,and all of children were divided into non-HPS and HPS group. The etiology, diagnosis, clinical features, biochemical, blood lipids,blood clotting,iron protein and NK cells of two groups were analyzed. Results ①100% of children with HPS had fever. 28 cases of children with non-HPS had fever, accounting for 71.79%. Fever rate of two groups was significant difference （χ^2= 4.62, P〈0.05）. ②The incidence of two cell lines reduction, high triglyeerides, low fibrinogen, high ferritin and low NK cell of HPS group were significantly higher than non-HPS group,the differences were significant （χ^2 =3.51- 6.36, P〈0.05）. ③Infectious diseases were mostly pathogen of HPS and non-HPS. The count of WBC, RBC, HB, PLT in HPS group were lower than non-HPS group, the differences were significant （t= 3.01 - 5.62, P〈0.05）. ④Visible Kawasa- ki disease and systemic juvenile rheumatoid arthritis were also major etiology. Conclusion Hemophagocytic phenomenon appear in many diseases, was not just restricted to HPS. It need to comprehensive evaluated the based on clinical symptoms and test results.

关 键 词：儿童 骨髓 噬血现象 噬血细胞综合征 

分 类 号：R557.4[医药卫生—血液循环系统疾病] R446.113[医药卫生—内科学]