Westerlund Per, Kurkus Jan, Segelmark Mårten
Department of Medicine, Blekinge Hospital, Karlshamn, Sweden.
Am J Kidney Dis. 2005 Jun;45(6):e97-9. doi: 10.1053/j.ajkd.2005.03.011.
Pure red cell aplasia (PRCA) is a rare, but important, complication of erythropoietin (EPO) replacement therapy in patients with renal disease. There is no consensus about the best way to treat this condition; however, recent reports indicated that immunosuppressive therapy is beneficial. We report a patient with EPO-induced PRCA treated with a regimen initially designed for antifactor VIII antibodies in patients with hemophilia. This regimen consists of immunoadsorption therapy using protein A columns, followed by oral prednisolone and single bolus infusions of intravenous immunoglobulin G and cyclophosphamide. Shortly after the course, a swift and rapid increase in reticulocyte count was evident; the patient became transfusion independent and has remained so during 2 years of follow-up. By means of this report, we wish to encourage others to consider this option when first-line treatments fail.
纯红细胞再生障碍性贫血(PRCA)是肾病患者促红细胞生成素(EPO)替代治疗中一种罕见但重要的并发症。对于治疗这种疾病的最佳方法尚无共识;然而,最近的报告表明免疫抑制治疗是有益的。我们报告了一名因EPO诱导的PRCA患者,采用最初为血友病患者抗因子VIII抗体设计的方案进行治疗。该方案包括使用蛋白A柱的免疫吸附治疗,随后口服泼尼松龙以及单次大剂量静脉输注免疫球蛋白G和环磷酰胺。疗程结束后不久,网织红细胞计数迅速明显增加;患者不再依赖输血,并且在2年的随访期间一直保持这种状态。通过本报告,我们希望鼓励其他人在一线治疗失败时考虑这种选择。