儿童系统性红斑狼疮相关纯红细胞再生障碍性贫血1例报告并文献复习

Pure red cell aplasia as presentation of systemic lupus erythematosus in children.

目的对文献报道的系统性红斑狼疮(SLE)相关的纯红细胞再生障碍性贫血(PRCA)患者的临床特征和实验室检查进行总结。方法对2003年2月中国协和医科大学血液病医院收治的1例儿童SLE相关PRCA及1966～2002年文献报道的资料完整的SLE相关PRCA患者26例(共27例)进行分析。结果70.4%(19/27)病例SLE和PRCA不能同时诊断。实验室检查无特异性。58.3%(14/24)患者泼尼松治疗有效,但常呈激素依赖性。结论SLE相关PRCA报道较少,临床表现和实验室检查缺少特异性,SLE相关PRCA治疗与PRCA的治疗相似。

Objective To review the clinical and laboratory features of the reported patients with systemic lupus erythematosus (SLE) and pure red cell aplasia (PRCA).Methods We identified cases reported during the years 1966~2002 by searching the MEDLINE literature.Results In most cases,SLE was diagnosed either before or after the diagnosis of PRCA.There were no significant differences in the laboratory findings.Prednisone did well in the majority of cases,but patients frequently remained steroid dependent.Conclusion The report of SLE associated PRCA is rare.There is no specificness in the clinical and laboratory features of SLE associated PRCA.Response to treatment of PRCA in patients with SLE is similar to those without SLE.