Prophylactic Eculizumab after Renal Transplantation in Atypical Hemolytic–Uremic Syndrome

Authors:
  • JHJohannes Hofer
    Johannes Hofer
  • GCGérard Cortina
    Gérard Cortina
  • WMWalter Mark
    Walter Mark
  • RWReinhard Würzner
    Reinhard Würzner
  • TJTherese C. Jungraithmayr
    Therese C. Jungraithmayr
  • GKGus Khursigara
    Gus Khursigara
  • KKKay O. Kliche
    Kay O. Kliche
  • WRWolfgang Radauer
    Wolfgang Radauer
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Abstract
Atypical hemolytic–uremic syndrome is a rare microangiopathic hemolytic condition characterized by thrombocytopenia and acute renal failure.1 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.2 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.3 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.