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首页> 外文期刊>BMC Nephrology >Eculizumab as salvage therapy for recurrent monoclonal gammopathy-induced C3 glomerulopathy in a kidney allograft
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Eculizumab as salvage therapy for recurrent monoclonal gammopathy-induced C3 glomerulopathy in a kidney allograft

机译:依库丽单抗作为挽救疗法用于同种异体肾移植术后复发性单克隆丙种球蛋白病诱发的C3肾小球病

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Monoclonal gammopathy causes several kinds of renal pathology. A rare and special form is monoclonal gammopathy-induced C3 glomerulopathy (MG-C3G). Like idiopathic C3G, MG-C3G frequently leads to end-stage renal disease. MG-C3G frequently recurs after renal transplantation, leading to graft failure in most of the patients. While there is some evidence for successful treatment of recurrent idiopathic C3 glomerulopathy with eculizumab after renal transplantation, nothing is known about its efficacy in the setting of recurrent MG-C3G. We report a patient with recurrent MG-C3G in a renal allograft that was successfully treated with eculizumab in addition to standard immunosuppression. He had early recurrence of MG-C3G 2?months after transplantation. His graft function successively declined despite high dose steroids and plasmapheresis. Only after therapy with three cycles of bortezomib and continuous therapy with eculizumab, his graft function stabilized. He was still in clinical remission after 28?months of follow-up without having experienced major infectious complications. Eculizumab may be a safe and effective treatment of recurrent MG-C3G. Because of the high and early recurrence risk, renal transplantation should be reviewed carefully for every individual patient. Subsequent hematopoietic stem cell transplantation may ameliorate long-term renal allograft survival. Eculizumab might serve as a bridging therapy until stem cell transplantation.
机译:单克隆丙种球蛋白病会导致几种肾脏病理。罕见的特殊形式是单克隆丙种球蛋白病诱发的C3肾小球病(MG-C3G)。像特发性C3G一样,MG-C3G经常导致终末期肾脏疾病。 MG-C3G在肾移植后经常复发,导致大多数患者的移植失败。尽管有一些证据表明在肾脏移植后使用依库丽单抗成功治疗了复发性特发性C3肾小球病,但对其在复发性MG-C3G中的疗效尚不清楚。我们报告了一名肾脏MG-C3G复发的患者,该患者已经成功用依库丽单抗和标准的免疫抑制疗法成功治疗了肾脏同种异体移植。移植后2个月,他的MG-C3G早期复发。尽管有大量的类固醇和血浆置换,他的移植物功能连续下降。仅在硼替佐米三个周期的治疗和依库丽单抗的连续治疗后,他的移植物功能才稳定。随访28个月后,他仍处于临床缓解期,未见重大感染并发症。依库丽单抗可能是复发性MG-C3G的安全有效治疗方法。由于高和早期复发的风险,应仔细检查每位患者的肾移植。随后的造血干细胞移植可改善肾脏异体移植的长期生存。在干细胞移植之前,依库丽单抗可能作为桥接疗法。

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