Successful treatment of anti-erythropoietin antibody-mediated pure red cell aplasia with low-dose prednisolone
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CASE REPORT
Successful treatment of anti-erythropoietin antibody-mediated pure red cell aplasia with low-dose prednisolone Kazunari Aoki • Yuichiro Ono • Sumie Tabata Akiko Matsushita • Takayuki Ishikawa
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Received: 12 July 2012 / Revised: 25 December 2012 / Accepted: 27 December 2012 / Published online: 26 January 2013 Ó The Japanese Society of Hematology 2013
Abstract The standard therapy for anti-erythropoietin (EPO) antibody-mediated pure red cell aplasia (PRCA) is cyclosporine (CyA) or prednisolone (PSL) 0.5–1.0 mg/kg. However, many patients with severe chronic kidney disease (CKD) and chronic heart failure cannot tolerate such an immunosuppressive regimen. An 86-year-old man with anemia related to CKD and chronic heart failure, who had received recombinant human erythropoietin subcutaneously, developed anti-EPO antibody-mediated PRCA. The patient was treated with CyA followed by PSL (1.0 mg/kg); however, he was unable to tolerate this drug regimen. The PSL dose was reduced to 0.2 mg/kg. Surprisingly, his reticulocyte count increased 3 months later, and RBC transfusion was no longer required. Low-dose PSL is a treatment option for patients with anti-EPO antibody-mediated PRCA who cannot tolerate CyA and PSL (0.5–1.0 mg/kg). Keywords Pure red cell aplasia Low-dose Prednisolone Anti-erythropoietin antibody
Introduction Anti-erythropoietin (EPO) antibody-mediated pure red cell aplasia (PRCA) is a rare but important complication in patients with anemia related to chronic kidney disease (CKD) who are treated with recombinant human
K. Aoki (&) Y. Ono S. Tabata A. Matsushita T. Ishikawa Department of Hematology and Clinical Immunology, Kobe City Medical Center General Hospital, 2-1-1 Minatojima-minami-machi, Chuo-ku, Kobe 650-0047, Japan e-mail: [email protected]
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erythropoietin (rhEPO) [1, 2]. The standard therapy for anti-EPO antibody-mediated PRCA is immunosuppressive treatment with cyclosporine (CyA) or prednisolone (PSL) 0.5–1.0 mg/kg [1]. However, many patients with anti-EPO antibody-mediated PRCA, who also have CKD and/or chronic heart failure, cannot tolerate this treatment. Here, we report the successful treatment of anti-EPO antibodymediated PRCA using low-dose PSL.
Case report An 86-year-old man developed CKD and chronic heart failure, which was followed-up by his family doctor. The CKD was probably due to chronic glomerulonephropathy, but a renal biopsy was not performed. His renal function gradually worsened, and he developed anemia related to CKD. RhEPO (epoetin-beta, 6,000 IU; EpoginÒ, Chugai, Tokyo, Japan) was administered subcutaneously once per week, and a substantial improvement was achieved (Fig. 1a). However, 5 months later, the anemia worsened, whereupon the patient was treated with a monthly subcutaneous dose of Epoetin-beta-pegol (150 lg; MirceraÒ, Chugai, Tokyo, Japan), which proved ineffective. The patient was then referred to our hospital. Laboratory results were as follows: hemoglobin, 6.1 g/dl; red blood cell count, 201 9 104/ll; mean corpuscular volume, 8
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