Renal Transplantation Under Prophylactic Eculizumab in Atypical Hemolytic Uremic Syndrome With CFH/CFHR1 Hybrid Protein

We report the first observation of successful kidney transplantation under pre‐emptive eculizumab treatment in a 7‐year‐old boy with atypical hemolytic uremic syndrome (aHUS) and a known hybrid CFH/CFHR1 gene, who was dependent on plasma therapy during the 3‐year dialysis period. The hybrid CFH/CFHR1 protein has an altered C3b/C3d binding, is incapable to protect cells from complement attack and is directly implicated in aHUS pathogenesis. There was no evidence of recurrence during the first 16‐month follow‐up period. We conclude that eculizumab alone, without plasma therapy (plasma infusion and/or plasma exchange), is sufficient to prevent recurrence of aHUS and to maintain long‐term graft function.