Department of Nephrology, The University of Hong Kong-Shenzhen Hospital, Shenzhen, China.
The First Clinical Medical College of Jinan University, Guangzhou, Guangdong, China.
CEN Case Rep. 2024 Dec;13(6):445-449. doi: 10.1007/s13730-024-00865-3. Epub 2024 Mar 25.
Recombinant human erythropoietin (rHuEPO) is commonly used to treat anemia associated with chronic kidney disease (CKD). EPO-induced Pure Red Cell Aplasia (PRCA) is a rare condition of profound anemia with EPO treatment. Upon finding the development of EPO-induced PRCA, the treatment requires immediate withdrawal of EPO therapy and initiate new treatments with immunosuppression or renal transplantation. Anti-EPO antibody assay is not always positive in EPO-induced PRCA. Here, we report a case on the sudden development of PRCA in a hemodialysis patient receiving EPO and how we treated the condition successfully with cyclosporine and subsequently maintained the hemoglobin with Roxadustat, a hypoxia-inducible factor prolyl hydroxylase inhibitor (HIF-PHI). Even though the anti-EPO antibody was negative by Enzyme Linked Immunosorbent Assay (ELISA) in our case, the clinical course, the markedly reduced reticulocyte count < 10,000/μL, the bone marrow (BM) biopsy revealing reduced erythroblasts, and its subsequent response to cyclosporine, were similar to EPO-induced PRCA. The clinical picture of EPO-induced PRCA, the limitation of the EPO-neutralizing antibody (Ab) assay, and treatment strategies were discussed.
重组人促红细胞生成素（rHuEPO）常用于治疗慢性肾脏病（CKD）相关贫血。EPO 诱导的纯红细胞再生障碍性贫血（PRCA）是一种罕见的 EPO 治疗后严重贫血的情况。发现 EPO 诱导的 PRCA 后，治疗需要立即停止 EPO 治疗，并开始新的免疫抑制或肾移植治疗。EPO 诱导的 PRCA 中的抗 EPO 抗体检测并不总是呈阳性。在这里，我们报告了一例接受 EPO 治疗的血液透析患者突然发生 PRCA 的病例，以及我们如何成功地使用环孢素治疗该疾病，随后使用罗沙司他（一种低氧诱导因子脯氨酰羟化酶抑制剂（HIF-PHI））维持血红蛋白。尽管我们的病例通过酶联免疫吸附试验（ELISA）检测抗 EPO 抗体为阴性，但临床病程、网织红细胞计数显著降低（<10,000/μL）、骨髓（BM）活检显示红细胞生成减少，以及随后对环孢素的反应与 EPO 诱导的 PRCA 相似。本文讨论了 EPO 诱导的 PRCA 的临床表现、EPO 中和抗体（Ab）检测的局限性以及治疗策略。
