成人斯蒂尔病继发巨噬细胞活化综合征的临床分析

发布时间：2018-03-02 01:35

本文关键词： 成人斯蒂尔病巨噬细胞活化综合征铁蛋白　出处：《上海交通大学学报(医学版)》2017年06期 　论文类型：期刊论文

【摘要】：目的·对成人斯蒂尔病(AOSD)合并巨噬细胞活化综合征(MAS)患者的临床资料进行分析总结,为该病的临床诊断和治疗提供依据。方法·收集上海交通大学医学院附属仁济医院2013年1月至2016年6月收治的42例AOSD患者,其中14例为AOSD合并MAS患者(MAS组),28例为性别、年龄匹配但未发生MAS的AOSD患者(非MAS组);对临床资料进行回顾性分析。结果·MAS组与非MAS组患者在性别构成、年龄、病程方面的差异均无统计学意义。与非MAS组比较,MAS组患者的病死率及皮疹、脾肿大、吞噬现象的发生率较高;血清谷丙转氨酶(ALT)、铁蛋白显著增高,纤维蛋白原(FDP)显著降低;在治疗方面,MAS组患者激素用量较大,甲氨蝶呤使用比例较低,丙种球蛋白使用比例较高,有2例接受依托泊苷治疗。结论·与非MAS组患者比较,MAS组患者的病死率较高;临床表现中皮疹、脾脏肿大、吞噬现象的发生率较高;血清铁蛋白、ALT显著升高,FDP显著降低。早期诊断积极治疗是改善预后的关键。
[Abstract]:Objective: to analyze and summarize the clinical data of adult patients with AOSD complicated with macrophage activation syndrome (MAS). Methods: from January 2013 to June 2016, 42 patients with AOSD were collected from Renji Hospital affiliated to Shanghai Jiaotong University Medical College. Age matched patients with AOSD without MAS (non-#en2# group); retrospective analysis of clinical data. Results: sex composition, age, age of patients in MAS group and non-#en4# group, The mortality, rash, splenomegaly, phagocytosis, serum alanine aminotransferase (alt), ferritin and fibrinogen (fibrinogen) were significantly increased in MAS group. In the treatment aspect, the dosage of hormone was higher, the proportion of methotrexate was lower, the proportion of gamma globulin was higher, and two patients were treated with etoposide. Conclusion: compared with the patients without MAS, the mortality of patients with MAS is higher. The incidence of rash, splenomegaly and phagocytosis was higher in clinical manifestations, while serum ferritin alt increased significantly and FDP decreased significantly. Early diagnosis and active treatment were the key to improve prognosis.
【作者单位】：上海交通大学医学院附属仁济医院风湿科;
【基金】：上海市教育委员会高峰高原学科建设计划(20152214) 国家重点研发计划项目(SQ2017YFSF090114) 国家自然科学基金(81373209)~~
【分类号】：R593.2

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