Division of Nephrology, Department of Medicine, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
Division of Vascular and Transplantation Surgery, Department of Surgery, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
Am J Case Rep. 2022 Jul 17;23:e935451. doi: 10.12659/AJCR.935451.
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/m2, 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.
纯红细胞再生障碍性贫血(PRCA)是终末期肾病(ESKD)贫血的一种罕见原因。它归因于重组人促红细胞生成素(rHuEPO)的给药。尽管免疫抑制是主要的治疗方法,但它的效果从 30%到 70%不等。据报道,ESKD 中的 PRCA 在肾移植后会改善。
一名 46 岁女性,因狼疮性肾炎导致 ESKD,在我们中心接受尿毒症治疗。她出现严重贫血。骨髓抽吸和活检显示红系前体细胞减少,符合 PRCA。由于她没有与她血型匹配的兄弟姐妹,因此 ABO 不相容的肾移植是一种治疗选择。她接受了一个脱敏方案,包括利妥昔单抗 375mg/m2、他克莫司、霉酚酸酯和泼尼松龙,在手术前 4 周进行,此外还在移植前一天进行了 3 次每两天一次的双重滤过血浆置换(DFPP),随后进行静脉注射免疫球蛋白(IVIG)和 1 次特异性免疫吸附(Glycosorb® B 柱)。她还接受了低剂量兔抗胸腺细胞球蛋白(rATG)(Thymoglobulin®)(总量 2.0mg/kg)。维持治疗包括他克莫司、霉酚酸酯和泼尼松龙。移植后几天,移植物功能恢复正常,她的 Hb 逐渐增加。
我们报告了一例罕见的 ESKD 患者接受 ABO 不相容肾移植后发生 PRCA 的病例。结果令人满意,贫血和肾功能完全纠正。