Gulzar Madeeha, Sabir Ali, Hamdani Muhammad Afzal, Saadia Asma
Department of Medicine, Shalamar Institute of Health and Sciences, Lahore, Pakistan.
Department of Pathology, Shalamar Institute of Health and Sciences, Lahore, Pakistan.
J Ayub Med Coll Abbottabad. 2018 Apr-Jun;30(2):289-292.
Macrophage activation syndrome (MAS) is a potentially lethal complication of chronic rheumatological conditions like ankylosing spondylitis, rheumatoid arthritis, and adult-onset Still's disease (AOSD). It is a multisystem inflammatory syndrome caused by immense cytokine release from activated lymphocytes and macrophages. We give an account of the incidence of a twenty years old Asian girl suffering from non-remitting fever and an evanescent rash for last ten weeks. Physical examination and laboratory work-up suggested high grade fever, pancytopenia, hyperferritinemia, hypertriglyceridemia, hypofibrinogenemia and impaired liver function. Bone marrow biopsy was also done. It showed active hemophagocytosis. She was diagnosed as a case of Macrophage Activation Syndrome associated with Adult Onset Still's disease. She was treated with high dose steroids and cyclosporine and recovered completely.
巨噬细胞活化综合征（MAS）是强直性脊柱炎、类风湿关节炎和成人斯蒂尔病（AOSD）等慢性风湿性疾病的一种潜在致命并发症。它是一种由活化淋巴细胞和巨噬细胞大量释放细胞因子引起的多系统炎症综合征。我们讲述了一名20岁亚洲女孩的发病情况，她在过去十周一直持续发热并伴有一过性皮疹。体格检查和实验室检查显示高热、全血细胞减少、高铁蛋白血症、高甘油三酯血症、低纤维蛋白原血症及肝功能受损。还进行了骨髓活检，结果显示有活跃的噬血细胞现象。她被诊断为与成人斯蒂尔病相关的巨噬细胞活化综合征病例。她接受了大剂量类固醇和环孢素治疗并完全康复。
