成人Still病继发巨噬细胞活化综合征(附4例报告)

目的总结成人Still病继发巨噬细胞活化综合征患者的临床特点。方法回顾我科2008年5月至2011年2月确诊4例成人Still病继发巨噬细胞活化综合征的临床资料并总结。结果1例在起病阶段．3例在复发时继发巨噬细胞活化综合征。临床特点包括稽留高热4例．皮疹2例、关节炎2例、浅表淋巴结肿大2例、脾肿大3例、肝肿大1例、外周血2系或全血细胞减少4例、肝损害3例、高甘油三酯3例、纤维蛋白原降低2例、骨髓细胞分类发现噬血现象4例。甲泼尼龙联合环孢素治疗3例．甲泼尼龙联合霉酚酸酯治疗1例。经治疗4例均缓解。结论持续性发热．血细胞减少．甘油三脂、乳酸脱氢酶升高以及纤维蛋白原降低是成人Still病继发巨噬细胞活化综合征的线索．骨髓细胞学检查有助于鉴别。糖皮质激素、环孢素及霉酚酸酯等免疫抑制剂治疗有效。

Macrophage activation syndrome in adult-onset Still's disease:a retrospective analysis of four patients

] Objective To analyze the clinical features of patients with macrophage activation syndrome (MAS) complicating adult onset Still's disease (AOSD). Methods The clinical data of four patients diagnosed with MAS and AOSD in our department from May 2008 to Jan 2011 were reviewed and summarized. Results One case occurred during the onset of AOSD and during the relapse in the rest of cases. The main symptoms were persistent fever (n=4), rash (n=2, arthritis(n=2), lymphoadenopathy(n=2), splenomegaly(n=3), heptome...