以血小板减少性紫癜为首发表现的儿童系统性红斑狼疮13例诊治分析

目的 总结儿童系统性红斑狼疮的诊治经验.方法 回顾性分析2004年1月至2009年6月我院收治的13例以急性免疫性血小板减少性紫癜为首发症状的小儿系统性红斑狼疮的诊断、治疗及转归情况.结果 13例初诊患儿经临床表现及骨髓检查诊断为急性免疫性血小板减少性紫癜，经用激素及静脉丙种球蛋白治疗效果欠佳，动态随诊免疫指标及其他临床表现，并根据血液系统改变特点和对激素的反应加用其他免疫抑制剂如环孢素A、霉酚酸酯及环磷酰胺等.13例患儿分别于2～24个月确诊为系统性红斑狼疮，加用其他免疫抑制剂后治疗效果满意.结论 对于血小板减少的患儿尤其是青春期女孩要注意动态监测抗核抗体及其他免疫学指标，警惕系统性红斑狼疮的可能.

Clinical analysis of systemic lupus erythematosus in 13 children with the first symptom of thrombocytopenic purpura

Objective To review the diagnosis and treatment of children with systemic lupus erythematosus. Methods 13 children of systemic lupus erythematosus with acute immune thrombocytopenic purpura as the first symptom were analyzed retrospectively, who were admitted from Jan 2004 to Jun 2009 in our hospital. Results Thirteen cases were diagnosed as acute immune thrombocytopenie purpura according to clinical manifestations and bone marrow examination results. Hormones and intravenous gamma globulin treatment were ineffective. The immune parameters and other clinical manifestations were monitored. According to the characteristics of blood system change and the response to hormone, other immunosuppressive agents such as cyclosporine A ,mycophenolate mofetil and cyclophosphamide were adminitrated. Thirteen children were diagnosed finally as systemic lupus erythematosus at 2 to 24 months from onset. Other immunosuppressive treatment was effective. Conclusion For children with thrombocytopenia, especially adolescent girls, we should pay attention to dynamic monitoring of anti-nuclear antibodies and other immunological parameters,for fear of the possibility of systemic lupus erythematosus.