Zeng P, Li F, Zeng H
Acta Reumatol Port. 2013 Jul-Sep;38(3):196-200.
Macrophage activation syndrome (MAS) is a severe and life-threatening complication of rheumatic disorders in children. We described a 9-year-old girl in whom MAS developed as a complication of systemic juvenile idiopathic arthritis (S-JIA) at onset with obvious hemophagocytosis presented in the marrow. She suffered from high fever and generalized rash subsequently joints swelling for two weeks before admission. Physical examination revealed mild cervical lymphadenopathy and hepatosplenomegaly. Laboratory findings were: abnormal liver enzymes, increased triglyceride and ferritin levels, anemia .Hyperplasia of hemophagocytic macrophages was remarkable in her bone marrow. Methylprednisolone and cyclosporin therapy resulted in clinical and laboratory improvement. It is unusual that hemophagocytosis presented in the marrow at onset of So-JIA without obvious abnormal coagulation profile, thrombocytopenia and leucopenia. It seemed that MAS may be occult at onset of SJIA. It may be integral to the pathogenesis of SJIA. The proper cyclosporine serum lever at the onset of MAS is as high as 200-300 ng/ml.
巨噬细胞活化综合征（MAS）是儿童风湿性疾病的一种严重且危及生命的并发症。我们描述了一名9岁女孩，她在系统性幼年特发性关节炎（S-JIA）发病时并发MAS，骨髓中出现明显的噬血细胞现象。入院前两周，她持续高热、全身皮疹，随后关节肿胀。体格检查发现轻度颈部淋巴结肿大和肝脾肿大。实验室检查结果显示：肝酶异常、甘油三酯和铁蛋白水平升高、贫血。她的骨髓中噬血细胞巨噬细胞增生明显。甲基强的松龙和环孢素治疗使临床症状和实验室检查结果得到改善。S-JIA发病时骨髓中出现噬血细胞现象，而凝血指标、血小板减少和白细胞减少无明显异常，这种情况并不常见。似乎MAS在S-JIA发病时可能隐匿存在。它可能是S-JIA发病机制的一个组成部分。MAS发病时合适的环孢素血清水平高达200 - 300 ng/ml。
