Verhelst David, Rossert Jérôme, Casadevall Nicole, Krüger Anne, Eckardt Kai-Uwe, Macdougall Iain C
Department of Nephrology, Tenon Hospital, Assistance Publique-Hôpitaux de Paris, and Pierre and Marie Curie University, Paris, France.
Lancet. 2004 May 29;363(9423):1768-71. doi: 10.1016/S0140-6736(04)16302-2.
Recombinant human erythropoietin is the standard treatment for anaemia related to chronic kidney disease, and its widespread use has been favoured by a very high therapeutic index. However, since 1998, more than 200 patients worldwide with chronic kidney disease treated in this way have developed neutralising antibodies to erythropoietin, causing pure red cell aplasia. We aimed to collate clinical and pathological features in patients unequivocally shown to have erythropoietin-induced pure red cell aplasia.
We retrospectively obtained data from the files of 47 patients with pure red cell aplasia. We assessed treatment and outcome of patients and defined recovery from pure red cell aplasia as an increase in reticulocyte counts to more than 20 000 per microL in patients who were no longer transfusion-dependent.
When patients developed pure red cell aplasia, all were receiving erythropoietin subcutaneously, and the product most typically prescribed was epoetin alfa (Eprex, Ortho Biotech). The median delay between start of erythropoietin treatment and occurrence of pure red cell aplasia was 11 months (IQR 7.5-14). Nine patients received no immunosuppressive treatment, and none of these recovered. Of 37 patients who received immunosuppressive therapy, 29 (78%) recovered. All six patients who received a kidney transplant recovered within 1 month, and recovery rates were between 56% and 88% in patients treated with corticosteroids, corticosteroids plus cyclophosphamide, or ciclosporin. No relapse of pure red cell aplasia happened after stopping immunosuppressive treatment, but no patient was rechallenged with erythropoietin.
Immunosuppressive treatment accelerates recovery from erythropoietin-induced pure red cell aplasia.
重组人促红细胞生成素是治疗慢性肾脏病相关性贫血的标准疗法，其极高的治疗指数使其得到广泛应用。然而，自1998年以来，全球有200多名接受此类治疗的慢性肾脏病患者产生了促红细胞生成素中和抗体，导致纯红细胞再生障碍性贫血。我们旨在梳理明确患有促红细胞生成素诱导的纯红细胞再生障碍性贫血患者的临床和病理特征。
我们回顾性地从47例纯红细胞再生障碍性贫血患者的病历中获取数据。我们评估了患者的治疗情况和预后，并将纯红细胞再生障碍性贫血的恢复定义为不再依赖输血的患者网织红细胞计数增加至每微升超过20000个。
患者发生纯红细胞再生障碍性贫血时均在接受皮下注射促红细胞生成素，最常开具的产品是阿法依泊汀（益比奥，奥多生物技术公司）。促红细胞生成素治疗开始至发生纯红细胞再生障碍性贫血的中位间隔时间为11个月（四分位间距7.5 - 14个月）。9例患者未接受免疫抑制治疗，无一例恢复。在37例接受免疫抑制治疗的患者中，29例（78%）恢复。所有6例接受肾移植的患者在1个月内恢复，接受皮质类固醇、皮质类固醇加环磷酰胺或环孢素治疗的患者恢复率在56%至88%之间。停用免疫抑制治疗后未发生纯红细胞再生障碍性贫血复发，但没有患者再次接受促红细胞生成素治疗。
免疫抑制治疗可加速促红细胞生成素诱导的纯红细胞再生障碍性贫血的恢复。
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