系统性红斑狼疮相关性重型再生障碍性贫血1例并文献复习

摘要

目的探讨分析系统性红斑狼疮（SLE）相关性重型再生障碍性贫血（SAA）的临床特征及治疗方案。方法回顾性分析收治的1例以血象减少起病的21岁女性SLE相关性SAA患者的症状、实验室检查、治疗及结局。结果患者经糖皮质激素治疗后血象继续下降，最终发展为SAA，同时并发狼疮脑病。相继给予大剂量甲基强的松龙、环磷酰胺、血浆置换等治疗后，狼疮脑病得到控制，但血象无恢复，入院后10d因多器官功能衰竭死亡。结论SLE相关性SAA缺乏确切有效的治疗方案，其治疗方式的有效性尚需进一步临床研究证实。%Objective To investigate the clinical characteristics and therapeutic methods of systemic lupus erythematosus(SLE) complicated with severe aplastic anaemia(SAA).Methods A 21-year-old woman with the initial symptom of pancytopenia was prospectively analyzed.Clinical symptoms,laboratory examinations,therapies and prognosis were observed for further study.Results She revealed no response to the initial glucocorticoid therapy and developed secondary SAA and lupus encephalopathy.Although high dose of methyl-prednisolone,cyclophosphamide pulses and plasmapheresis were inducted,pancytopenia didn’t revived,with lupus encephalopathy controlled.Finally,she died of multiple organ failure 10 days after being admitted into the hospital. Conclusion Effective therapies are lacking in SLE complicated with SAA and more clinical studies are needed to conifrm the effectiveness of various therapeutic strategies.