A 46-Year-Old Thai Woman with Secondary Acquired Pure Red Cell Aplasia Due to Treatment with Recombinant Erythropoietin While on Dialysis for End-Stage Renal Disease Who Recovered Following ABO-Incompatible Kidney Transplantation

Abstract

Patient: Female, 46-year-old Final Diagnosis: Pure red cell aplasia (PRCA) undergoing ABO-incompatible kidney transplantation Symptoms: End-stage kidney disease (ESKD) with anemia Medication:— Clinical Procedure: — Specialty: Nephrology Objective: Rare disease Background: Pure red cell aplasia (PRCA) is an uncommon cause of anemia in end-stage kidney disease (ESKD). It is attributed to recombinant human erythropoietin (rHuEPO) administration. Although immunosuppression is the mainstay therapy, its effectiveness varies from 30% to 70%. PRCA in ESKD has been reported to improve following kidney transplantation. Case Report: A 46-year-old woman with ESKD secondary to lupus nephritis was treated for uremia at our center. She developed severe anemia. Bone marrow aspiration and biopsy revealed a reduction of erythroid precursors, consistent with PRCA. Because she had no sibling’s blood group matched with her, ABO-incompatible kidney transplantation was an option for treatment. She underwent a desensitization protocol consisting of rituximab 375 mg/m 2 , tacrolimus, mycophenolate mofetil, and prednisolone 4 weeks before surgery, in addition to 3 sessions of double-filtration plasmapheresis (DFPP) every other day followed by intravenous immunoglobulin (IVIG) and 1 session of specific immunoadsorption (Glycosorb ® B column) at pre-transplant day −1. She also received low-dose rabbit anti-thymocyte globulin (rATG) (Thymoglobulin ® ) (total 2.0 mg/kg). Maintenance therapy included tacrolimus, mycophenolate mofetil, and prednisolone. Allograft function normalized a few days after transplantation and her Hb gradually increased. Conclusions: We report a rare case of PRCA in a patient with ESKD undergoing ABO-incompatible kidney transplantation. The outcome was satisfactory, with complete correction of anemia and kidney function.

HLA-B*46:01:01:01 and HLA-DRB1*09:01:02:01 are associated with anti-rHuEPO-induced pure red cell aplasia

“…The Thai ESA registry reported that the incidence of anti-rHuEPO-induced PRCA in Thai patients was at least 1.7 per 1000 patient years 18 . This incidence is much higher than the incidence of anti-rHuEPO-induced PRCA in western countries 2 , 4 , 9 – 12 , 18 – 20 . Our previous data 4 and other groups 3 , 7 suggested the role of HLA-DRB1*09-DQB1*03:09 and HLA DRB1*12:02 as risk factors for anti-rHuEPO-induced PRCA.…”

Case report: Dynamic antibody monitoring in a case of anti-recombinant human erythropoietin-mediated pure red cell aplasia with prolonged course after kidney transplantation

“…A retrospective study collected anti-EPO antibody-mediated PRCA cases in French and German from 1998 to 2003, which found that there were 6 cases in total and they all got recovered one month after undergoing kidney transplant ( 6 ). We also reviewed other case reports published afterward and found that 6 (85.7%) out of 7 ESRD cases with anti-EPO antibody-mediated PRCA got recovered with Hb levels above 100 g/L within 3 months after kidney transplantation ( 12 – 15 ). Only one case reported partial recovery at 28 months after transplantation with persistent anti-EPO antibodies but independent of blood transfusion ( 13 ).…”