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目的分析巨噬细胞活化综合征(MAS)的临床特点、治疗及转归,以提高其诊治水平。方法回顾性分析广州市儿童医院2003年1月至2006年8月所收治的9例MAS患者的临床资料。结果9例患儿中7例为男性,2例为女性,年龄5个月～12岁。临床表现以长期发热为主,骨髓噬血现象,血细胞减少,可伴随有浅表淋巴结肿大、肝脾肿大、肝功能损害、脂代谢异常,糖皮质激素及免疫抑制剂治疗有效,其中2例出现急性呼吸窘迫综合征(ARDS),2例出现多器官功能衰竭(MOF),2例死亡。结论MAS病情凶险,预后差,提高对该病的认识,及早治疗十分重要。
Objective To analyze the clinical features, treatment and prognosis of macrophage activation syndrome (MAS) in order to improve its diagnosis and treatment. Methods The clinical data of 9 patients with MAS admitted to Guangzhou Children’s Hospital from January 2003 to August 2006 were retrospectively analyzed. Results Of the 9 children, 7 were male and 2 were female, ranging in age from 5 months to 12 years. The main clinical manifestations of long-term fever, hemophagocytic bone marrow, cytopenia, may be accompanied by superficial lymph nodes, hepatosplenomegaly, liver damage, dyslipidemia, glucocorticoid and immunosuppressive agents effective treatment, of which 2 Cases of acute respiratory distress syndrome (ARDS), 2 cases of multiple organ failure (MOF), 2 patients died. Conclusion MAS is dangerous and the prognosis is poor. It is very important to improve the understanding of the disease and early treatment.