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Pure red cell aplasia and anti-erythropoietin antibodies in patients on hemodialysis: a report of two cases and a literature review

机译:血液透析患者的纯红细胞发育不全和抗促红细胞生成素抗体:两例报告并文献复习

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Introduction: Anemia is a frequent multifactorial complication of CKD seen in patients on dialysis derived mainly from impaired erythropoietin (EPO) production. A less common cause of anemia in individuals with CKD is pure red cell aplasia (PRCA) secondary to the production of anti-EPO antibodies. Objective: This paper aimed two describe two cases of PRCA secondary to the production of anti-EPO antibodies including choice of treatment, patient progression, and a literature review. Materials: This study included the cases of two patients with CKD on hemodialysis with severe anemia in need of specific investigation and management. Results: Patient 1 with CKD secondary to hypertension treated with EPO for 7 months showed persistent decreases in hemoglobin (Hb) levels despite the subcutaneous administration of increasing doses of EPO; the patient required recurring blood transfusions. Workup and imaging tests were negative for the main causes of anemia in individuals with CKD on dialysis. Patient 2 with CKD secondary to adult polycystic kidney disease had been taking EPO for 2 years. The patient developed severe abrupt anemia the month he was started on HD, and required recurring transfusions to treat the symptoms of anemia. Workup and imaging findings were inconclusive. Specific laboratory tests confirmed the patients had anti-EPO antibodies. After six months of immunosuppressant therapy (corticosteroids + cyclosporine) the patients were stable with Hb 9.0 g/dl. Conclusion: PRCA is a rare condition among patients on dialysis treated with rhEPO and should be considered as a possible cause of refractory anemia. Treating patients with PRCA may be challenging, since the specific management and diagnostic procedures needed in this condition are not always readily available.
机译:简介:贫血是透析患者中​​常见的CKD多因素并发症,主要来自促红细胞生成素(EPO)产生受损。 CKD患者贫血的一种较不常见原因是继抗EPO抗体产生后的纯红细胞发育不良(PRCA)。目的:本文针对两例描述了继抗EPO抗体产生后继发的PRCA的两个案例,包括治疗选择,患者病情进展和文献复习。材料:本研究包括两名患有严重贫血的血液透析CKD患者,需要进行具体调查和处理。结果:尽管皮下给药增加了EPO剂量,但因EPO治疗7个月的高血压继发性CKD患者1表现出血红蛋白(Hb)水平持续下降;患者需要反复输血。透析后CKD患者贫血的主要原因是检查和影像学检查阴性。继发于成人多囊肾疾病的CKD患者2已接受EPO治疗2年。患者开始进行HD手术后一个月就发生了严重的突然性贫血,需要反复输血以治疗贫血症状。检查和影像学检查结果尚无定论。特定的实验室测试证实患者具有抗EPO抗体。经过六个月的免疫抑制剂治疗(皮质类固醇+环孢霉素),患者稳定,Hb> 9.0 g / dl。结论:在rhEPO治疗的透析患者中​​,PRCA是一种罕见病,应被认为是难治性贫血的可能原因。治疗PRCA患者可能具有挑战性,因为在这种情况下所需的特定管理和诊断程序并不总是容易获得。

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