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目的 描述成人巨噬细胞活化综合征的临床特点,以提高对此病的认识.方法 对13例成人综合征患者的临床表现、实验室检查、治疗及转归的临床资料进行分析.结果 最常见的症状和体征为发热、关节痛、脾大、黄疸及肝大.最常见的实验室检查异常为转氨酶异常、高铁蛋白血症、2系以上血细胞减少、高甘油三酯血症及凝血功能异常.患者的平均铁蛋白水平为10 237μg/L.所有患者均使用糖皮质激素治疗,7例联合免疫抑制剂治疗.5例患者死亡,8例好转(其中2例出现原发疾病的复发).结论 成人巨噬细胞活化综合征是风湿免疫性疾病的一种严重并发症,发病时症状不典型,如出现不能解释的高热、血细胞减少、铁蛋白显著升高、甘油三酯升高等表现等,应警惕巨噬细胞活化综合征的出现,应积极完善检查以及时诊断.

Objective To evaluate clinical feature of adult Macrophage Activition Syndrome in order to improve our recognition of it. Methods Clinical data of 13 patients with Macrophage Activition Syndrome were analyzed. Results Fever, arthritis, splenomegaly, jaundice and hepatomegaly were the most common symptoms or signs seen in adult MAS patients. Increased liver enzymes, hyperferritinemia, hypocytosis, hypertriglyceridemia and abnormal coagulation were the most common abnormal laboratory examination. Average ferritin level of the patients was 10237 μg/L. All patients received glucocorticosteroid. 7 patients received additional immune sup-pression treatment. 5 patients died, and 8 patients got remission (2 patiens relapsed in the course of disease). Conclusion Adult Macrophage Activition Syndrome is a life-threatening complication of rheumatic disease and has no typical symptom at onset. When there axe unexplained high fever, hypoeytosis, hyperferritinemia and hy-pertriglyceridemia in rheumatic disease, we should take bone marrow examination to diagnosis the disease as soon as possible.