Recovery from anti-recombinant-human-erythropoietin associated pure red cell aplasia in end-stage renal disease patients after renal transplantation

Kearkiat Praditpornsilpa 2 3 Somphon Buranasot 2 3 Narudee Bhokaisuwan 0 2 Yingyos Avihingsanon 2 3 Trairak Pisitkul 2 3 Talerngsak Kansanabuch 2 3 Somchai Eiam-Ong 2 3 Sauwaluck Chusil 2 3 Tanin Intarakumtornchai 1 2 Kriang Tungsanga 2 3 0 Laboratory Medicine, Department of Medicine, Faculty of Medicine, Chulalongkorn University Hospital , Bangkok , Thailand 1 Division of Hematology 2 Division of Nephrology, Department of Medicine, Faculty of Medicine, Chulalongkorn University Hospital , Bangkok , Thailand 3 Division of Nephrology Introduction Case report Switch from alpha to beta r-HuEPO PRC PRC PRC PRC PRC PRC 3 u 3 u 2 u 2 u 2 u 4 u Hgb (g/dl) Alpha and beta-r-HuEPO dose (u/kg/wk) Bone marrow biopsy Cyclophosphamide 1 mt 2 mts 3 mts 4 mts Prednisolone Transplantation Switch from beta to alpha r-HuEPO PRC 3 u PRC 3 u PRC 3 u Hb (g/dl) Beta and alpha-r-HuEPO dose (u/kg/wk) Bone marrow biopsy 2mts 3mts Transplantation Fig. 1. Continued. Hb (g/dl) Hb (g/dl) Transplantation Transplantation Beta-r-HuEPO dose (u/kg/wk) PRC 3 u PRC 3 u PRC 3 u PRC 3 u PRC 6 u PRC 6 u PRC 8 u Bone marrow biopsy 1mt 2mts 3mts 4mts Alpha-r-HuEPO dose (u/kg/wk) PRC 2 u PRC 3 u PRC 2 u PRC 2 u PRC 4 u PRC 6 u PRC 4 u PRC 8 u Bone marrow biopsy 1mt 2mts 3mts 4mts aimed for 1500 ng/ml 2 h post-dose for 1 month and 15001300 ng/ml for 13 months. Following kidney transplantation, the haemoglobin (Hgb) levels in all patients returned to more than 10.0 g/dl at 12, 8, 12, 10 weeks post-transplantation, respectively. The reticulocyte count increased after the transplantation (Figure 2). At 4 week post-transplant, anti-r-HuEPO was negative. Patient A Patient B Patient C Patient D 1mt 2mts 3mts 4mts Transplantation Four patients displayed remission of anti-r-HuEPO associated PRCA after renal transplantation. The average time to Hgb >10.0 g/dl was 10.5 weeks. None of the patients required a blood transfusion post-transplantation. Treatment of non-anti-r-HuEPO-associated PRCA with steroids, cytotoxic agents, intravenous immunoglobulin (IVIG) or cyclosporine [9] yields response rates ranging from 30 to 55%. Presently, the optimal therapy for anti-r-HuEPO associated PRCA is uncertain; previous data have shown that failures are common. Also there is no report of a spontaneous recovery of anti-r-HuEPO associated PRCA. In our series, decadurabolin, an anabolic steroid previously used to treat renal anaemia in pre-r-HuEPO era, failed to raise the Hgb of one patient. Furthermore, high doses of prednisolone for 12 weeks and cyclophosphamide for 8 weeks did not improve the anaemia, this patient required continued transfusions. A switch from the alpha to the beta, or vice versa, forms of r-HuEPO also showed no significant increase in responsiveness to treatment. Interestingly, all patients displayed HLA DR B1*9, an allele present in 8.7% of the general Thai population. Considering the low incidence of anti-r-HuEPO associated PRCA and the prevalence of HLA B1*9 in this subset of patients, we speculate that there may be Conflict of interest statement. We (Praditpornsilpa K, Buranasot S, Bhokaisuwan N, Avihingsanon Y, Pisitkul T, Kansanabuch T, Eiam-Ong S, Intarakumtornchai T, Tungsanga K) declare no conflict of interest in this study. (...truncated)