肾移植后补体介导的血栓性微血管病:是否应终身使用 C5 抑制剂治疗?

IF 2.3 4区 医学 Q2 UROLOGY & NEPHROLOGY Nephron Pub Date : 2024-04-14 DOI:10.1159/000538826
Pilar Musalem, C. Pedreros-Rosales, H. Müller-Ortiz, Carlos Gutiérrez-Navarro, J. D. Carpio
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引用次数: 0

摘要

补体介导的血栓性微血管病(CM-TMA)是一种罕见且危及生命的并发症,可发生在肾移植受者身上,其潜在诱因包括免疫抑制药物。对于这类患者,使用 C5 抑制剂(C5i)治疗 CM-TMA 的最佳治疗方法和疗程仍在研究之中。我们介绍了一例 38 岁女性患者的病例,她曾患有 IgA 肾病,接受了先期活体相关肾移植,移植后 7 年患上了 CM-TMA。使用雷珠单抗治疗后,血液学反应迅速,血小板计数趋于稳定。补体功能测试的连续测量结果和临床稳定性为停止 C5i 治疗提供了指导。该病例凸显了肾移植受者 CM-TMA 管理的复杂性,尤其是在确定 C5i 治疗的适当持续时间方面。由于缺乏既定的停药方案,因此有必要根据临床和实验室稳定性、无补体基因变异和连续补体功能测试采取个性化方法。有必要开展进一步的前瞻性研究,以确定在这一特殊患者群体中监测和安全停用 C5i 治疗的最佳策略。该病例强调了个体化治疗在肾移植后 CM-TMA 管理中的重要性,并为潜在的停药标准提供了启示。
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COMPLEMENT-MEDIATED THROMBOTIC MICROANGIOPATHY AFTER KIDNEY TRANSPLANT: SHOULD TREATMENT WITH C5 INHIBITOR BE LIFELONG?
Complement-mediated thrombotic microangiopathy (CM-TMA) is a rare and life-threatening complication that can occur in kidney transplant recipients, with various potential triggers including immunosuppressive medications. The optimal management and duration of treatment with C5 inhibitors (C5i) for CM-TMA in this patient population remain areas of ongoing investigation. We present the case of a 38-year-old female with a history of IgA nephropathy who underwent preemptive living-related kidney transplantation and subsequently developed CM-TMA seven years post-transplant. Treatment with ravulizumab led to a rapid hematologic response and stabilized platelet counts. Serial measurements of complement functional tests and clinical stability guided the discontinuation of C5i therapy. The case highlights the complexity of managing CM-TMA in kidney transplant recipients, particularly in determining the appropriate duration of C5i therapy. The absence of an established protocol for discontinuation necessitates a personalized approach based on clinical and laboratory stability, absence of complement gene variants, and serial complement functional tests. Further prospective investigations are warranted to define the optimal strategies for monitoring and safely discontinuing C5i therapy in this unique patient population. This case underscores the importance of individualized care in the management of CM-TMA post-kidney transplantation, offering insights into potential criteria for therapy discontinuation.
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来源期刊
Nephron
Nephron UROLOGY & NEPHROLOGY-
CiteScore
5.00
自引率
0.00%
发文量
80
期刊介绍: ''Nephron'' comprises three sections, which are each under the editorship of internationally recognized leaders and served by specialized Associate Editors. Apart from high-quality original research, ''Nephron'' publishes invited reviews/minireviews on up-to-date topics. Papers undergo an innovative and transparent peer review process encompassing a Presentation Report which assesses and summarizes the presentation of the paper in an unbiased and standardized way.
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