Anti-erythropoietin antibody-related pure red cell aplasia (anti-EPO PRCA) is a severe complication in patients who receive erythropoiesis-stimulating agents for nephrogenic anemia. The standard therapy is withdrawl of EPO and immunosuppression. Here, we present successful treatment of anti-EPO PRCA with roxadustat, a hypoxia-inducible factor prolyl hydroxylase inhibitor. A 39-year-old woman with end-stage renal disease received recombinant human erythropoietin (rhEPO) subcutaneously. Unfortunately, she developed anti-EPO PRCA and her hemoglobin dropped continuously, whereas she rejected immunosuppressive therapy. The patient failed to achieve spontaneous hematologic recovery with cessation of rhEPO alone, and she became transfusion dependent. Thus, she accepted our advice to try roxadustat for nephrogenic anemia. Surprisingly, after starting roxadustat treatment, her reticulocyte and hemoglobin improved gradually. Four months later, the bone marrow aspiration smear demonstrated a return to normal in erythroid cells. Besides, her anti-erythropoietin antibody converted to negative. All in all, this case reveals the potential effect of roxadustat on anti-EPO PRCA.
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