尿毒症合并EPO抗体介导的纯红细胞再生障碍性贫血1例临床报告

目的:提高对尿毒症合并EPO抗体介导的纯红细胞再生障碍性贫血的认识。方法:患者男59岁,2型糖尿病13年,血肌酐升高2年余,乏力1年余,多次查血红蛋白提示中重度贫血,药物治疗效果不佳,需输红细胞治疗。初步诊断:糖尿病肾病尿毒症肾性贫血。入院后完善血常规、网织红细胞绝对值、铁蛋白及转铁蛋白饱和度、血生化、大便常规,ANA、ANCA、血尿免疫固定电泳、EPO抗体及骨髓穿刺活检等检查。结果:予以rHuEPO、铁剂及激素、免疫抑制剂、输血等药物治疗后患者仍反复贫血,骨穿及骨髓活检提示纯红细胞再生障碍性贫血,结合血清EPO抗体阳性,网织红细胞绝对值低下,诊断考虑EPO抗体介导的纯红细胞再生障碍性贫血(PRCA),予以激素及免疫制剂治疗,患者继发严重肺部感染,最终停止激素及免疫抑制剂治疗,仅靠输血改善贫血。后经罗沙司他(100~120 mg tiw po)治疗,贫血得以纠正,目前Hb维持在110~120 g/L,乏力明显改善。结论:尿毒症并发贫血时,治疗效果不佳需要积极查找贫血原因,EPO抗体介导的PRCA是其原因之一,此例患者经罗沙司他治疗,血红蛋白维持相对好的水平,乏力改善,提示罗沙司他治疗该患者贫血有效。

Is iron overload in alcohol-related cirrhosis mediated by hepcidin?

In this case report we describe the relationship between ferritin levels and hepcidin in a patient with alcohol-related spur cell anemia who underwent liver transplantation.We demonstrate a reciprocal relation-ship between serum or urinary hepcidin and serum ferritin,which indicates that inadequate hepcidin production by the diseased liver is associated with elevated serum ferritin.The ferritin level falls with increasing hepcidin production after transplantation.Neither inflammatory indices(IL6)nor erythropoietin appear to be related to hepcidin expression in this case.We suggest that inappropriately low hepcidin production by the cirrhotic liver may contribute substantially to elevated tissue iron stores in cirrhosis and speculate that hepcidin replacement in these patients may be of therapeutic benefi t in the future.