自身免疫性溶血性贫血继发纯红细胞再生障碍危象：1例报告及文献复习

Autoimmune hemolytic anemia secondary to pure red cell aplasia crisis:one case report and literatures review

目的:报告1例自身免疫性溶血性贫血(autoimmune hemolytic anemia,AIHA)继发纯红细胞再生障碍(pure red cell aplasia,PRCA)危象的病例。方法:对1例AIHA继发PRCA危象患者的临床资料进行回顾性分析,并复习相关文献。结果:患者,女,59岁,因发热伴一过性白细胞、血小板减少,进行性重度贫血8天就诊。辅助检查:重度贫血,肝功能异常,尿胆原(+),血红蛋白尿,乳酸脱氢酶增高,骨穿示红系造血停滞。临床诊断为AIHA继发PRCA危象,病毒感染,肝功能异常。予泼尼松+静脉丙球(IVIG)、输红细胞悬液等治疗。病程3周时脱离输血,肝功能好转。结论:AIHA继发PRCA危象少见且严重。及时去除诱因,予糖皮质激素+IVIG+红细胞悬液输注支持治疗可获痊愈。

Objective： To report one case of autoimmune hemolytic anemia （AIHA） secondary to pure red cell aplasia （PRAC） crisis. Methods： The clinical data from the patient who experienced AIHA secondary to PRCA crisis was retrospectively analyzed and the related literatures were reviewed. Results： A 59-year-old female visited doctor for the complaint about fever with transient neutropenia, thrombocytopenia, and progressive development to severe anemia for 8 days. In the course of the disease she had abnormal liver function, increase of urobilinogen, hemoglobinuria and lactate dehydrogenase （LDH）, and bone marrow aspiration showed erythroid stasis. She was diagnosed as viral infection, AIHA secondary to PRCA crisis, liver function abnormality, and then treated with prednisone, intravenous immunoglobulin （IVIG）, red cell suspension transfusion and supportive treatment. She had no need for transfusion after 3 weeks treatment. Conclusion： We speculate that the AIHA secondary to PRCA crisis is a rare but serious condition by reviewing the related literatures. Treatment with glucocorticoid, IVIG and transfusions may help to recovery.