Andrade Jason, Taylor Paul A, Love Janet M, Levin Adeera
Department of Medicine, University of British Columbia, St Paul's Hospital, Vancouver BC, Canada.
Nephrol Dial Transplant. 2005 Nov;20(11):2548-51. doi: 10.1093/ndt/gfi018. Epub 2005 Jul 26.
We report a 3-year case history that describes a 78-year-old woman with recurrent transfusion-dependent pure red cell aplasia (PRCA) secondary to recombinant epoetin use that was responsive to immunosuppressant therapy. The patient had kidney disease of unknown aetiology (estimated glomerular filtration rate of 13 ml/min/1.73 m2) and was not on dialysis. After 16 months of therapy with subcutaneous Eprex, she developed anti-erythropoietin antibody-confirmed PRCA and was started on high dose prednisone (50 mg per day). Within 5 months, the patient's serum was clear of antibodies and, under the cover of low dose prednisone (5-7.5 mg per day), therapy with a different erythropoiesis-stimulating compound (Aranesp) was initiated due to persistent fatigue and anaemia. At 3 months of therapy, the serum anti-erythropoietin antibodies remained negative and, due to the patient's requests, and after discussion, prednisone therapy was discontinued. Unfortunately, 3 months after cessation of prednisone, a recurrence of PRCA was confirmed by the development of profound anaemia and reappearance of anti-erythropoietin antibodies in the patient's serum. High dose prednisone (50 mg per day) was reinstituted, whereupon, 2 months later, antibodies were again confirmed to be negative. This case report demonstrates the responsiveness of PRCA to simple immunosuppressive therapy, and the ability to introduce different erythropoiesis-stimulating agents in the presence of such therapy. It appears that there may be problems associated with discontinuation of immunosuppressive therapy in the presence of sustained erythropoiesis-stimulating agent therapy in those in whom the condition has occurred previously.
我们报告了一例3年的病例史，该病例描述了一名78岁女性，因使用重组促红细胞生成素继发反复输血依赖的纯红细胞再生障碍性贫血（PRCA），对免疫抑制治疗有反应。患者患有病因不明的肾脏疾病（估计肾小球滤过率为13 ml/min/1.73 m2），未进行透析。皮下注射益比奥治疗16个月后，她出现了抗促红细胞生成素抗体确诊的PRCA，并开始使用高剂量泼尼松（每天50 mg）。5个月内，患者血清中抗体消失，由于持续疲劳和贫血，在低剂量泼尼松（每天5 - 7.5 mg）的掩护下，开始使用另一种促红细胞生成刺激化合物（阿法依泊汀）治疗。治疗3个月时，血清抗促红细胞生成素抗体仍为阴性，经讨论并应患者要求，停用泼尼松治疗。不幸的是，停用泼尼松3个月后，患者出现严重贫血，血清中再次出现抗促红细胞生成素抗体，确诊PRCA复发。重新使用高剂量泼尼松（每天50 mg），2个月后，抗体再次被确认为阴性。本病例报告证明了PRCA对简单免疫抑制治疗的反应性，以及在这种治疗存在的情况下引入不同促红细胞生成刺激剂的能力。在先前发生过这种情况的患者中，在持续使用促红细胞生成刺激剂治疗的情况下停用免疫抑制治疗似乎可能存在问题。
