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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 summarize the clinical features and laboratory tests of patients with systemic lupus erythematosus (SLE) -related pure red cell aplasia (PRCA) reported in the literature. Methods One case of SLE-related PRCA in children with hematology hospital of Peking Union Medical College in February 2003 and 26 cases (27 cases) of complete SLE-related PRCA reported in the literature from 1966 to 2002 were analyzed. Results 70.4% (19/27) cases of SLE and PRCA can not be diagnosed simultaneously. Laboratory tests are nonspecific. Prednisone was effective in 58.3% (14/24) patients, but was often hormone-dependent. Conclusions SLE-related PRCA is rarely reported, and clinical manifestations and laboratory tests lack specificity. SLE-related PRCA treatment is similar to PRCA treatment.