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巨噬细胞活化综合征 被引量:4

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摘要 巨噬细胞活化综合征(MAS)是幼年特发性关节炎全身型(soJIA)严重而致命的并发症,及时诊断和处理是必要的。本文综述了soJIA并MAS的触发因素和发病机制、临床表现、实验室和组织病理检查、诊断以及治疗。典型的MAS表现为持续发热,肝脾和淋巴结大,急剧的血沉降低和全血细胞减少,严重的肝功能损害,凝血障碍及神经系统受累。血沉下降和高血清铁蛋白血症是MAS最为突出的特点之一;骨髓和其他器官发现大量具有显著噬血活性的非肿瘤性组织细胞增生。环孢素A是治疗的首选药物。
出处 《实用儿科临床杂志》 CAS CSCD 北大核心 2006年第3期179-182,共4页 Journal of Applied Clinical Pediatrics
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