Lacreta Gabriela, Bucharles Sérgio Gardano Elias, Sevignani Gabriela, Riella Miguel Carlos, Nascimento Marcelo Mazza do
Universidade Federal do Paraná, Hospital de Clínicas, Serviço de Nefrologia, Curitiba, PR, Brasil.
Fundação Pró Renal, Curitiba, PR, Brasil.
J Bras Nefrol. 2019 Jan-Mar;41(1):145-151. doi: 10.1590/2175-8239-jbn-2018-0054. Epub 2018 Aug 23.
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.
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.
This study included the cases of two patients with CKD on hemodialysis with severe anemia in need of specific investigation and management.
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.
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.
贫血是慢性肾脏病(CKD)常见的多因素并发症,见于接受透析的患者,主要源于促红细胞生成素(EPO)生成受损。CKD患者贫血的一个较不常见原因是继发于抗EPO抗体产生的纯红细胞再生障碍性贫血(PRCA)。
本文旨在描述两例继发于抗EPO抗体产生的PRCA病例,包括治疗选择、患者病情进展及文献综述。
本研究纳入了两名接受血液透析的CKD患者,他们患有严重贫血,需要进行特定的检查和管理。
患者1因高血压继发CKD,接受EPO治疗7个月,尽管皮下注射的EPO剂量不断增加,但血红蛋白(Hb)水平持续下降;该患者需要反复输血。检查和影像学检查未发现透析CKD患者贫血的主要原因。患者2因成人多囊肾病继发CKD,已接受EPO治疗2年。该患者在开始血液透析的当月出现严重的急性贫血,需要反复输血以治疗贫血症状。检查和影像学检查结果不明确。特定实验室检查证实患者存在抗EPO抗体。经过6个月的免疫抑制治疗(皮质类固醇+环孢素),患者病情稳定,Hb>9.0g/dl。
PRCA在接受重组人促红细胞生成素(rhEPO)治疗的透析患者中是一种罕见疾病,应被视为难治性贫血的可能原因。治疗PRCA患者可能具有挑战性,因为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。