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低剂量泼尼松龙治疗抗促红细胞生成素抗体介导的纯红细胞再生障碍有效。

Successful treatment of anti-erythropoietin antibody-mediated pure red cell aplasia with low-dose prednisolone.

机构信息

Department of Hematology and Clinical Immunology, Kobe City Medical Center General Hospital, 2-1-1 Minatojima-minami-machi, Chuo-ku, Kobe 650-0047, Japan.

出版信息

Int J Hematol. 2013 Feb;97(2):272-4. doi: 10.1007/s12185-013-1258-3. Epub 2013 Jan 26.

DOI:10.1007/s12185-013-1258-3
PMID:23355260
Abstract

The standard therapy for anti-erythropoietin (EPO) antibody-mediated pure red cell aplasia (PRCA) is cyclosporine (CyA) or prednisolone (PSL) 0.5-1.0 mg/kg. However, many patients with severe chronic kidney disease (CKD) and chronic heart failure cannot tolerate such an immunosuppressive regimen. An 86-year-old man with anemia related to CKD and chronic heart failure, who had received recombinant human erythropoietin subcutaneously, developed anti-EPO antibody-mediated PRCA. The patient was treated with CyA followed by PSL (1.0 mg/kg); however, he was unable to tolerate this drug regimen. The PSL dose was reduced to 0.2 mg/kg. Surprisingly, his reticulocyte count increased 3 months later, and RBC transfusion was no longer required. Low-dose PSL is a treatment option for patients with anti-EPO antibody-mediated PRCA who cannot tolerate CyA and PSL (0.5-1.0 mg/kg).

摘要

抗红细胞生成素 (EPO) 抗体介导的纯红细胞再生障碍性贫血 (PRCA) 的标准治疗方法是环孢素 (CyA) 或泼尼松龙 (PSL) 0.5-1.0mg/kg。然而,许多患有严重慢性肾脏病 (CKD) 和慢性心力衰竭的患者无法耐受这种免疫抑制方案。一位 86 岁的男性因 CKD 和慢性心力衰竭导致贫血,曾接受过皮下重组人促红细胞生成素治疗,随后发生了抗 EPO 抗体介导的 PRCA。该患者接受了 CyA 随后是 PSL(1.0mg/kg)治疗;然而,他无法耐受该药物方案。PSL 剂量减少至 0.2mg/kg。令人惊讶的是,他的网织红细胞计数在 3 个月后增加,不再需要输血。对于不能耐受 CyA 和 PSL(0.5-1.0mg/kg)的抗 EPO 抗体介导的 PRCA 患者,低剂量 PSL 是一种治疗选择。

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Pure red cell aplasia due to antibody against erythropoietin in hemodialysis patients.血液透析患者中因抗促红细胞生成素抗体导致的纯红细胞再生障碍。
J Nephropathol. 2017 Jan;6(1):25-29. doi: 10.15171/jnp.2017.05. Epub 2016 Aug 22.
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Targeting EPO and EPO receptor pathways in anemia and dysregulated erythropoiesis.

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Antiepoetin antibody-related pure red cell aplasia: successful remission with cessation of recombinant erythropoietin alone.抗促红素抗体相关纯红细胞再生障碍性贫血:单独停用重组促红素后缓解。
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N Engl J Med. 2002 Feb 14;346(7):469-75. doi: 10.1056/NEJMoa011931.
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