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目的探讨血清铁蛋白(SF)在噬血细胞性淋巴组织细胞增生症(HLH)患儿诊断和疗效评价中的作用。方法回顾性分析2005年5月-2009年5月郑州大学第一附属医院儿科收治的40例初诊HLH住院患儿临床资料,应用放射免疫分析法定期检测40例HLH患儿SF水平,并选取同期本院门诊体检儿童中与研究对象年龄、性别相匹配的健康儿童35例作为健康对照组,HLH患儿全部采用HLH-04治疗方案,根据早期治疗效果将HLH患儿分为完全缓解(CR)组、部分缓解(PR)组、未缓解(NR)组,比较各组治疗前后SF水平。结果 1.HLH组治疗前血清SF均≥500μg.L-1,其中32例(80%)>1000μg.L-1,且治疗前HLH组SF水平显著高于健康对照组(P<0.01);2.HLH组早期治疗后CR(23例)和PR(8例)的患儿SF水平显著下降,与治疗前比较差异有统计学意义(P<0.01),NR组9例患儿治疗前后SF水平比较差异无统计学意义(P>0.05);3.CR患儿23例中7例复发,复发时SF再次增高,与CR时SF水平比较差异有统计学意义(P<0.01)。结论 1.SF水平升高对HLH具有可靠的诊断价值,SF水平越高,诊断HLH的可靠性越大;2.监测HLH患儿SF水平有助于病情判断,其值变化与病情活动度有关,且对临床疗效有评价作用。
Objective To investigate the role of serum ferritin (SF) in the diagnosis and therapeutic evaluation of hemophagocytic lymphohistiocytosis (HLH). Methods The clinical data of 40 newly diagnosed HLH hospitalized children admitted to the First Affiliated Hospital of Zhengzhou University from May 2005 to May 2009 were retrospectively analyzed. The serum levels of SF in 40 children with HLH were detected by radioimmunoassay (RIA) 35 healthy children matched with the age and gender of the study subjects were selected as healthy control group. HLH children were all treated with HLH-04. According to the early treatment effect, HLH children were divided into complete remission (CR) Group, partial remission (PR) group, untreated (NR) group. The levels of SF in each group were compared before and after treatment. Serum SF of ≥500μg.L-1 in HLH group before treatment was 32% (80%)> 1000μg.L-1, SF level in HLH group before treatment was significantly higher than that in healthy control group (P <0.01); SF level of children with CR (23 cases) and PR (8 cases) after early treatment in HLH group was significantly lower than that before treatment (P <0.01), and SF in 9 children with NR before and after treatment (P> 0.05) .3. Among the 23 cases with CR, 7 cases relapsed, the SF increased at the time of recurrence, and the difference was statistically significant (P <0.01) with that at the time of CR. SFL level of HLH has a reliable diagnostic value, the higher the SF level, the greater the reliability of the diagnosis of HLH; 2. SF levels in children with HLH contribute to the judgment of the disease, and its value changes with the degree of disease activity , And evaluate the clinical efficacy.