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目的:总结成人结缔组织病并巨噬细胞活化综合征临床特点,提高对本病的认识。方法:回顾分析2002年1月至2009年2月收治的9例结缔组织病并巨噬细胞活化综合征患者的可能病因、临床表现、治疗及专归。结果:9例患者,男3例,女6例。临床表现为持续高热、肝脾和淋巴结肿大、黄疸、血液系统受累。实验室检查提示有全血细胞减少、肝功能异常、乳酸脱氢酶增高、高铁蛋白血症、严重低纤维蛋白原血症,部分凝血功能异常,骨髓涂片可见噬红细胞现象。治疗上予大剂量甲泼松龙联合环孢霉素可能有一定的疗效。结论:巨噬细胞活化综合征可见于成人结缔组织病,并是其一个严重的、致死的并发症。大部分患者疗效不佳,预后差。
OBJECTIVE: To summarize the clinical features of adult connective tissue disease and macrophage activation syndrome and to raise awareness of the disease. Methods: Nine patients with connective tissue disease and macrophage activation syndrome who were admitted to our hospital from January 2002 to February 2009 were retrospectively analyzed. The possible etiologies, clinical manifestations, treatments and specializations were retrospectively analyzed. Results: Nine patients, 3 males and 6 females. Clinical manifestations of persistent high fever, liver and spleen and lymph nodes, jaundice, blood system involvement. Laboratory tests suggest pancytopenia, abnormal liver function, increased lactate dehydrogenase, hyperferricin, severe hypofibrinogenemia, partial coagulation dysfunction, bone marrow smear shows red blood cell phenomenon. Treatment of large doses of methylprednisolone combined cyclosporin may have a certain effect. Conclusion: Macrophage activation syndrome is found in adult connective tissue disease and is a serious and lethal complication. Most patients poor efficacy, poor prognosis.