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依库珠单抗治疗一名非典型溶血性尿毒症综合征成年患者的经验。

Eculizumab experience in an adult patient with atypical hemolytic uremic syndrome.

作者信息

Sari Funda, Inci Ayça, Karakus Volkan, Yilmaz Banu, Sarikaya Metin, Olmaz Refik, Kurtoglu Erdal

机构信息

Department of Nephrology, Antalya Training and Research Hospital, Antalya, Turkey.

Department of Hematology, Antalya Training and Research Hospital, Antalya, Turkey.

出版信息

Saudi J Kidney Dis Transpl. 2017 Mar-Apr;28(2):388-391. doi: 10.4103/1319-2442.202783.

Abstract

Atypical hemolytic-uremic syndrome is a disease characterized by nonimmune hemolytic anemia, thrombocytopenia, and renal failure. In this study, we present a case of a patient with atypical hemolytic-uremic syndrome treated successfully with eculizumab. A 20-year-old female was admitted with clinical signs of atypical hemolytic syndrome. The laboratory findings were as follows: hemoglobin 9.2 g/dL, platelet count 18 × 103/μL, creatinine 4.69 mg/dL, schistocytes were in peripheral blood smear, lactate dehydrogenase 2080 U/L, and emergency plasmapheresis procedure with fresh frozen plasma were initiated. The patient was anuric within 12 h of her admittance. ADAMTS13 activity was normal. Her mother's cousin developed acute rejection immediately after receiving a renal transplant and died two months later. As she did not respond to the treatment and considering her family history, eculizumab was initiated which resulted in platelet counts starting to rise on day 5, and the patient no longer needed dialysis after 22 days.

摘要

非典型溶血尿毒综合征是一种以非免疫性溶血性贫血、血小板减少和肾衰竭为特征的疾病。在本研究中,我们报告了一例成功使用依库珠单抗治疗的非典型溶血尿毒综合征患者。一名20岁女性因非典型溶血性综合征的临床症状入院。实验室检查结果如下:血红蛋白9.2g/dL,血小板计数18×10³/μL,肌酐4.69mg/dL,外周血涂片可见裂体细胞,乳酸脱氢酶2080U/L,并启动了用新鲜冷冻血浆进行的紧急血浆置换程序。患者入院后12小时内无尿。ADAMTS13活性正常。她母亲的表弟在接受肾移植后立即发生急性排斥反应,并在两个月后死亡。由于她对治疗无反应并考虑到其家族史,开始使用依库珠单抗治疗,结果血小板计数在第5天开始上升,22天后患者不再需要透析。

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