Lim Lay-Cheng
Department of Hematology, Singapore General Hospital, Singapore, 169608, Singapore.
Hematology. 2005 Jun;10(3):255-9. doi: 10.1080/10245330400026154.
Acquired pure red cell aplasia (PRCA) is a rare condition. Traditionally it has been described in association with various etiologies such as parvovirus B19 infection, auto-immune disorders and drugs. Immunologically mediated PRCA is by far the commonest cause in adults, particularly since 1998, when a marked increased incidence of PRCA was noted in chronic renal failure patients receiving subcutaneous (SC) recombinant erythropoietin (rEpo). Typically these patients had been given erythropoietin for correction of anemia of renal failure and subsequently present with severe transfusion dependent anemia. Most cases were associated with SC administration of human serum albumin (HSA) free erythropoieitin alfa product (Eprex). Early recognition and withdrawal of erythropoietin therapy is essential. Treatment with immunosuppressive therapy, particularly in conjunction with renal transplant results in good response with resolution in the majority of cases. The pathogenesis is related to interaction of multiple factors such as formulation change and improper storage leading to increased immunogenicity of the recombinant product. The incidence peaked in 2001 and 2002, subsequently dropping considerably from 2003. This can be explained by the institution of measures such as more stringent handling and storage conditions, improvements in formulation of HSA free Eprex and switch to intravenous (IV) administration for Eprex in dialysis patients. The evidence to date on this condition is summarized in this review.
获得性纯红细胞再生障碍性贫血(PRCA)是一种罕见疾病。传统上,它被描述为与多种病因相关,如细小病毒B19感染、自身免疫性疾病和药物。免疫介导的PRCA是目前成人中最常见的病因,特别是自1998年以来,当时在接受皮下(SC)重组促红细胞生成素(rEpo)的慢性肾衰竭患者中,PRCA的发病率显著增加。典型的情况是,这些患者曾接受促红细胞生成素治疗以纠正肾衰竭贫血,随后出现严重的依赖输血的贫血。大多数病例与皮下注射不含人血清白蛋白(HSA)的促红细胞生成素α产品(益比奥)有关。早期识别并停用促红细胞生成素治疗至关重要。免疫抑制治疗,特别是与肾移植联合使用,在大多数病例中可产生良好反应并使病情缓解。其发病机制与多种因素的相互作用有关,如制剂改变和储存不当导致重组产品免疫原性增加。发病率在2001年和2002年达到峰值,并在2003年之后大幅下降。这可以通过采取更严格的处理和储存条件、改进不含HSA的益比奥制剂以及透析患者改用静脉注射(IV)益比奥等措施来解释。本综述总结了迄今为止关于这种疾病的证据。