Prophylactic eculizumab after renal transplantation in atypical hemolytic-uremic syndrome.

摘要

Atypical hemolytic-uremic syndrome is a rare microangiopathic hemolytic condition characterized by thrombocytopenia and acute renal failure. The prognosis for patients with atypical hemolytic-uremic syndrome with a factor H mutation is poor; 60% have end-stage renal disease or die within a year. The expected rate of graft failure due to recurrent atypical hemolytic-uremic syndrome among patients with a factor H or factor I mutation is 80% within 1 to 2 years. Here, we describe a child with atypical hemolytic-uremic syndrome and a known factor H mutation who had normal renal function 1 year after high-risk kidney transplantation with prophylactic dosing of the terminal complement inhibitor eculizumab.