Alpay Nadir, Ozçelik Umit
Department of Nephrology, İstanbul Aydın University Training and Research Hospital, İstanbul, Turkey.
Department of General Surgery, Istanbul Aydın University Training and Research Hospital, İstanbul, Turkey.
Transplant Proc. 2019 Sep;51(7):2295-2297. doi: 10.1016/j.transproceed.2019.01.157. Epub 2019 Aug 7.
Hemolytic uremic syndrome (HUS) is characterized by microangiopathic anemia, thrombocytopenia, and acute kidney injury. HUS is mostly associated with diarrhea (90%). However, 10% of cases are not associated with diarrhea and are thus called as atypical HUS (aHUS); these cases are usually caused by dysregulation of the complement system. Eculizumab, a monoclonal antibody against C5, is the drug of choice for treating aHUS. Herein we aimed to present 8 cases of renal transplantation performed on patients with aHUS.
A total of 8 patients who had been diagnosed with aHUS between the years 2012 to 2018 were enrolled and underwent transplantations. All patients received induction treatment, standard immunosuppresive treatment (tacrolimus, mycophenolic acid, prednisolone), and eculizumab. Eculizumab was administered at a dosage of 900 mg/wk for the first month and 1200 mg every 2 weeks thereafter. Patients were followed up and recorded in terms of demographic features, serum creatinine, lactate dehydrogenase, acute rejection episodes, and allograft outcomes.
Mean age was 34 ± 8 years (Male/Female: 6/2). One of the patients had a second transplantation. Median hemodialysis vintage (25%-75% interquartile range) was 37 (9-63) months. Four patients had pretransplant plasmapheresis and 2 patients had posttransplant plasmapheresis. Induction treatment was ATG in 7 patients, and basiliximab was used only in 1 patient. The median follow-up period was 25 (13-59) months. Mean serum creatinine levels were 1.9 ± .6, 1.2 ± .7, and 1 ± .1 mg/dL for the first day, first month, and last values, respectively. Mean lactate dehydrogenase levels were 286 ± 203, 239 ± 27, and 218 ± 86 U/L for first day, first month, and last values, respectively. None of the patients had an acute rejection episode. Currently, all patients have functioning allografts.
Patients with aHUS may be transplanted successfully with eculizumab with good allograft outcomes.
溶血性尿毒症综合征（HUS）的特征为微血管病性贫血、血小板减少和急性肾损伤。HUS大多与腹泻相关（90%）。然而，10%的病例与腹泻无关，因此被称为非典型HUS（aHUS）；这些病例通常由补体系统失调引起。依库珠单抗是一种抗C5单克隆抗体，是治疗aHUS的首选药物。在此，我们旨在介绍8例接受肾移植的aHUS患者。
纳入2012年至2018年间共8例诊断为aHUS的患者并接受移植。所有患者均接受诱导治疗、标准免疫抑制治疗（他克莫司、霉酚酸、泼尼松龙）和依库珠单抗。依库珠单抗首月剂量为900mg/周，此后每2周1200mg。对患者进行随访，并记录其人口统计学特征、血清肌酐、乳酸脱氢酶、急性排斥反应发作情况及移植肾结局。
平均年龄为34±8岁（男/女：6/2）。其中1例患者进行了二次移植。血液透析中位时间（四分位间距25%-75%）为37（9-63）个月。4例患者在移植前进行了血浆置换，2例患者在移植后进行了血浆置换。7例患者诱导治疗使用抗胸腺细胞球蛋白，仅1例患者使用巴利昔单抗。中位随访期为25（13-59）个月。首日、首月及末次血清肌酐平均水平分别为1.9±0.6、1.2±0.7和1±0.1mg/dL。首日、首月及末次乳酸脱氢酶平均水平分别为286±203、239±27和218±86U/L。所有患者均未发生急性排斥反应。目前，所有患者的移植肾均功能良好。
aHUS患者使用依库珠单抗进行移植可能成功，移植肾结局良好。
