儿童巨噬细胞活化综合征与其他噬血细胞综合征的临床比较分析

Comparative analysis of childhood macrophage activation syndrome and other hemophagocytic syndrome

目的观察儿童巨噬细胞活化综合征（MAS）与其他噬血细胞综合征（HPS）的临床特点，探究两者在临床特点、诊断、治疗及预后上的不同之处。方法收集2006年1月至2009年3月所有HPS患儿共36例，分为MAS组13例，其他HPS23例。运用t检验、x^2检验及Fisher精确概率法统计两者在临床特点、实验室指标、治疗方法及预后情况上的差异。结果MAS患儿年龄明显大于其他HPS患儿[（7．7±1．3）岁和（2．6±0．5）岁，t=3．899，P=0．004]；而性别分布上差异无统计学意义。临床表现上MAS患儿中枢神经系统受累（69％和13％，P=0．001）、循环系统受累（23％和9％，P=0．047）及泌尿系统受累（38％和9％，P=0．033）更为常见且临床症状更为严重。在实验室检查上，MAS患儿血清铁蛋白的增高、红细胞沉降率（ESR）的动态变化更为明显，其中铁蛋白增高[（9703±9819）μg/L和（4569±1396）μg／L，t=2．854，P=0．015]、ESR动态变化[（53±32）ram／1h和（20±14）mm／1h，t=2．708，P=0．020]。结论MAS与其他HPS在发病年龄、病因、各系统的累及、临床表现的严重程度、一些实验室检查变化及治疗方法上有所差别。

Objective To compare the clinical features, diagnosis, treatment and prognosis between macrophages activation syndrome （MAS） and other hemophagocytic syndrome （HPS）. Methods Thirty-six children with HPS were identified at Nanjing Children＇s Hospital during January 2006 to March 2009. They could be classi-fied into MAS group （13 patients） and other HPS group （23 patients）. All relevant clinical features, laboratory data, treatments and outcomes were analyzed with t test, x^2 test and Fisher＇s exact test. Results Patients with MAS tended to be elder than those with other HPSs [（7.7±1.3） years vs （2.6±0.5） years, t=3.899, P=0.004]. There was no difference in gender distribution. In MAS cases, the central nervous system （69% vs 13%, P=-0.001 ）, circulatory system （23% vs 9%, P=0.047） and the urinary system （38% vs 9%, P=0.033 ） were usually involved, The clinical symptoms of MAS were more sever than other HPS. Serum ferritin [（9703＋9819） p,g/L vs （4569±1396） μg/L, t=2.854, P=0.015] and erythrocyte sedimentation rate （ESR） [（53±32） mm/1 h vs （20±14） mm/l h, t=2.708, P=0.020] changed more obviously in MAS cases compared with other HPS. Conclusion Childhood MAS is different from other HPS in texans of age, etiology, clinical manifestations, laboratory tests and treatments.