Department of Nephrology, Kyoto Yamashiro General Medical Center, 1-27 Kizuekimae, Kizugawa, Kyoto, 619-0214, Japan.
Department of Nephrology, Kyoto Prefectural University of Medicine, 465 Kajii-cho, Kamigyo-ku, Kyoto, 602-8566, Japan.
BMC Nephrol. 2020 Jul 14;21(1):275. doi: 10.1186/s12882-020-01934-2.
Erythropoietin-stimulating agents (ESAs) are used to treat anemia in patients with chronic kidney disease, enabling maintenance of stable hemoglobin levels and eliminating the need for multiple transfusions. Epoetin-beta pegol (C.E.R.A.) is a continuous erythropoietin receptor activator created by integrating a large methoxy-polyethylene-glycol-polymer chain into the erythropoietin molecule, which provides it with a longer half-life. On rare occasions, cases of antibody-mediated pure red cell aplasia (PRCA) related to ESAs are reported. They are characterized by abrupt onset of severe transfusion-dependent anemia, despite ESA therapy. We herein report a case of antibody-mediated PRCA in a dialysis patient receiving C.E.R.A.
A 44-year-old man with end-stage renal failure had been receiving continuous ambulatory peritoneal dialysis for 2 years. C.E.R.A. was administered subcutaneously as a sole ESA once a month at the hospital since 4 years ago for the treatment of renal anemia and his hemoglobin level was well controlled at 12 g/dl. From 10 months before diagnosis, however, his hemoglobin level suddenly declined, necessitating frequent transfusions. Based on the results of a bone marrow examination and detection of anti-C.E.R.A. antibodies, the patient was diagnosed with antibody-mediated PRCA. After successful elimination of the antibodies using oral prednisolone plus cyclosporine, the patient was re-administrated C.E.R.A. intravenously, as there are few reports of antibody-mediated PRCA related to ESA using that administration route. He responded to the C.E.R.A., and his anemia dramatically improved, eliminating the need for blood transfusions.
This is the first reported case of recovery from an antibody-mediated PRCA with C.E.R.A. after its re-administration following a reversal of the antibody. It has been suggested that the additional large pegylation chain makes C.E.R.A. less likely to trigger antibody generation than other ESAs. Following successful treatment of antibody-mediated PRCA using immunosuppressive therapy, C.E.R.A. can be re-administered intravenously to treat renal anemia.
促红细胞生成素刺激剂（ESAs）用于治疗慢性肾脏病患者的贫血，使稳定的血红蛋白水平得以维持，并消除了多次输血的需要。聚乙二醇化促红素-β（C.E.R.A.）是一种通过将大分子甲氧基聚乙二醇聚合物链整合到促红细胞生成素分子中而产生的连续促红细胞生成素受体激活剂，这使其半衰期更长。在极少数情况下，会报告与 ESAs 相关的抗体介导的纯红细胞再生障碍性贫血（PRCA）病例。它们的特征是尽管接受了 ESA 治疗，但突然出现严重的依赖输血的贫血。在此，我们报告了一名接受 C.E.R.A.治疗的透析患者发生抗体介导的 PRCA 的病例。
一名 44 岁的终末期肾病男性患者，2 年前开始接受持续非卧床腹膜透析。4 年前，他开始每月在医院接受一次皮下 C.E.R.A.治疗肾性贫血，血红蛋白水平控制良好，为 12g/dl。然而，从诊断前 10 个月开始，他的血红蛋白水平突然下降，需要频繁输血。根据骨髓检查结果和抗 C.E.R.A.抗体的检测结果，该患者被诊断为抗体介导的 PRCA。在使用口服泼尼松龙加环孢素成功消除抗体后，该患者重新接受了静脉内 C.E.R.A.治疗，因为很少有关于使用该给药途径的 ESA 相关抗体介导的 PRCA 的报道。他对 C.E.R.A.有反应，贫血显著改善，无需输血。
这是首例报道的 C.E.R.A.在抗体逆转后重新给药并从抗体介导的 PRCA 中恢复的病例。据推测，额外的大 PEG 化链使 C.E.R.A.不太可能引发抗体产生，而其他 ESAs 则更容易引发抗体产生。在使用免疫抑制疗法成功治疗抗体介导的 PRCA 后，可以重新给予静脉内 C.E.R.A.以治疗肾性贫血。
