COMPLEMENT-MEDIATED THROMBOTIC MICROANGIOPATHY AFTER KIDNEY TRANSPLANT: SHOULD TREATMENT WITH C5 INHIBITOR BE LIFELONG?

血栓性微血管病 伊库利珠单抗 补语(音乐) 医学 微血管病 肾移植 补体成分5 补体系统 肾移植 内科学 免疫学 化学 内分泌学 互补 糖尿病 抗体 生物化学 疾病 基因 表型
作者
Pilar Musalem,Cristian Pedreros‐Rosales,Hans Müller-Ortiz,Carlos Gutiérrez-Navarro,J. Daniel Carpio
标识
DOI:10.1159/000538826
摘要

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 7 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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