Remarkable response to roxadustat in a case of anti-erythropoietin antibody-mediated pure red cell aplasia
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LETTER TO THE EDITOR
Remarkable response to roxadustat in a case of anti-erythropoietin antibody-mediated pure red cell aplasia Ke Wan 1 & Yafei Yin 2 & Zimian Luo 2 & Jianzhao Cheng 1 Received: 9 June 2020 / Accepted: 9 September 2020 # The Author(s) 2020
Dear Editor, Pure red cell aplasia (PRCA) is a rare but serious complication occurring in patients with chronic kidney disease (CKD) who are treated with recombinant human erythropoietin (rHu-EPO). Patients typically develop an abrupt onset of severe transfusion-dependant anemia. Merely withdrawing rHu-EPO is not sufficient to reverse the condition, and immunosuppressive treatment with corticosteroids, cyclophosphamide, or ciclosporin only partly corrects anemia [1]. Roxadustat, an oral inhibitor of hypoxia-inducible factor prolyl hydroxylase, has showed efficacy in correcting anemia in patients with CKD who are receiving or not receiving dialysis [2, 3]. The present report is the first to describe a case of PRCA related to rHu-EPO whose hemoglobin reversed dramatically after switching to roxadustat treatment. A 49-year-old man developed chronic renal failure due to obstructive nephropathy. He did not have other comorbidities. In November 2018, his serum creatinine level reached 631 μmol/L and hemoglobin (Hb) concentration decreased to 64 g/L. He received peritoneal dialysis and was administered 6000 units of rHu-EPO subcutaneously twice a week. Anemia improved substantially, his Hb level increased to about 100 g/L for 5 months. In June 2019, his Hb suddenly fell to 46 g/L without evidence of bleeding, infection, and hemolysis. In response, rHu-EPO was increased to 6000 units 3 times a week. In the following 5 months, he was admitted to
Ke Wan and Yafei Yin contributed equally and should be considered cofirst authors. * Yafei Yin [email protected] 1
Department of Nephrology, Xiangtan Central hospital, City, Xiangtan, Hunan Province, China
2
Department of Hematology, Xiangtan Central hospital, City, Xiangtan 411100, Hunan Province, China
hospital monthly for transfusion of red blood cells, his anemia improved temporarily after transfusion, and the levels of Hb at each admission fluctuated between 32 and 49 g/L. In late October, further laboratory examinations were performed. He had Hb concentration of 49 g/L, reticulocyte count of 3.4 × 109/L. The level of serum EPO was below detectable limits (normal value 4.3–29.0 IU/L). Bone marrow aspirate showed severe hypoplasia of the erythroid line with hyperplasia of the granulocytic and megakaryocytic lines, normal ratio of blast cells, and no morphologic dysplasia. Assays for anti-DNA Ab, anti-nuclear Ab, hepatitis B antigen, and parvovirus B19 DNA were not detected. Computed tomography (CT) of the chest and abdomen found nothing abnormal. Anti-EPO antibody was checked by ELISA and turned out to be positive. Collectively, these findings pointed to a diagnosis of anti-EPO antibody-mediated PRCA. On November 7, 2019, rHu-EPO administrations were interrupted and he was prescribed oral roxadustat (100 mg thrice we
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