Late onset of de novo atypical hemolytic–uremic syndrome presented on a simultaneous pancreas and kidney transplant recipient successfully treated with eculizumab

摘要

Atypical hemolytic–uremic syndrome (aHUS) is an extremely rare, genetic, chronic, and progressive inflammatory disease1 caused by defects in complement system. These defects result in systemic thrombotic microangiopathy involving damage to multiple organ systems including renal dysfunction.1–7 Genetic mutations have been detected in around 50% of the reported cases. Regarding renal transplant patients, expanded criteria donors, infection by cytomegalovirus or BK, use of CNI or m-TOR inhibitors and antibody-mediated rejection (AMR) have been related to de novo post-transplant aHUS.8 Graft failure is reported in 60–90% of patients within 1 year.10 Eculizumab is a monoclonal antibody that binds to C5 complement protein avoiding the formation of the cell membrane attack complex.6,7 We report herein a case of late onset of de novo post-transplant aHUS on a simultaneous pancreas and kidney recipient with severe systemic manifestations, without presenting acute graft rejection, successfully treated with limited doses of eculizumab remaining stable after one year of follow-up.