Efficacy of eculizumab in the treatment of recurrent atypical hemolytic-uremic syndrome after renal transplantation.

摘要

Atypical hemolytic-uremic syndrome (aHUS) is a microangiopathic throm-botic syndrome with a prominent renal involvement. So far, plasma exchange has been the usual treatment although it currently only has a category III indication (not clearly indicated) according to American Society for Apheresis (1). Despite the adult onset, 50% cases are related to mutations in regulatory complement proteins (complement factor H [CFH], factor I, membrane cofactor protein, CFH-related proteins, and C4b-binding protein) (2).