Fraer Mony, Campbell Adam, Sawaya B Peter
Division of Nephrology, Bone and Mineral Metabolism, University of Kentucky, Lexington, USA.
Semin Dial. 2006 May-Jun;19(3):251-4. doi: 10.1111/j.1525-139X.2006.00163.x.
Reported cases of pure red cell aplasia (PRCA) from the administration of erythropoietin (EPO)-alpha molecule in the United States are rare, and the optimal treatment is still unknown. We present a patient with end-stage renal disease (ESRD) who became hyporesponsive and later unresponsive to EPO-alpha treatment a few months after initiation of hemodialysis. A comprehensive anemia examination was negative while the patient became transfusion dependent. The diagnosis of EPO-alpha-induced PRCA was confirmed by bone marrow biopsy, by undetectable serum EPO levels following the administration of a large dose of EPO-alpha, and by documenting the presence of EPO-neutralizing antibodies. Administration of cyclosporine A in addition to prednisone enabled the patient to become transfusion and EPO independent. This case further documents the possible occurrence of PRCA with EPO-alpha administration in the United States and reaffirms the potential beneficial effect of cyclosporine A.
在美国,报告的因使用α-促红细胞生成素(EPO)分子而导致纯红细胞再生障碍性贫血(PRCA)的病例很少见,最佳治疗方法仍不明确。我们报告了一名终末期肾病(ESRD)患者,该患者在开始血液透析几个月后对α-EPO治疗反应低下,随后无反应。全面的贫血检查结果为阴性,而患者变得依赖输血。通过骨髓活检、大剂量α-EPO给药后血清EPO水平检测不到以及记录到EPO中和抗体的存在,确诊为α-EPO诱导的PRCA。除泼尼松外,给予环孢素A使患者不再依赖输血和EPO。该病例进一步证明了在美国使用α-EPO可能发生PRCA,并再次证实了环孢素A的潜在有益作用。