Korean J Transplant.  2022 Nov;36(Supple 1):S53. 10.4285/ATW2022.F-1708.

Eculizumab therapy for recurrent atypical hemolytic uremic syndrome after kidney transplantation in atypical hemolytic uremic syndrome: a case report

• Kim K
• Yang SJ
• Chong SY
• Lee YH

• KMID: 2535257
• DOI: http://doi.org/10.4285/ATW2022.F-1708

Abstract

Atypical hemolytic uremic syndrome (aHUS), non-Shiga-toxin-HUS, is a rare cause of end stage renal disease. Recently, four regulatory proteins of the complement alternative pathway, complement factor H (CFH), membrane cofactor protein (MCP or CD46), factor I (CFI) and thrombomodulin (THBD) and two proteins of the C3 convertase, C3 and factor B (CFB), had a role in the pathogenesis of aHUS. All patients with a clinical diagnosis of primary aHUS are eligible for treatment with a complement inhibitor like eculizumab. The outcome of kidney transplantation in aHUS is poor and is largely predicted by the underlying genetic alteration. But the lowest incidence of recurrence was observed in patients with MCP mutations. We report a case in which eculizumab was used as a treatment for relapse of aHUS in a patient who underwent a kidney transplant after being diagnosed with renal failure due to aHUS (MCP mutation). A 34-year-old male patient was diagnosed as aHUS with MCP muta-tion, and received treatment with eculizumab, dialysis and plasma exchange. Despite therapy, his renal function did not recover. So, he underwent a living donor kidney transplantation from his mother. She had also MCP mutation that was not revealed. Five months after the kidney transplant, he developed recurrent aHUS and suspicious antibody-mediated rejection (AMR). So, He was treated with rituximab and plasmapheresis for AMR and resumed treatment with eculizumab for recurrent aHUS. Now he is well-followed up with controlled disease status.