Eculizumab-sparing Protocol in Renal Transplantation of Patients with Atypical Hemolytic Uremic Syndrome in a Resource-limited Setting

We devised and applied eculizumab sparing protocols in renal transplantation in patients with atypical hemolytic uremic syndrome (aHUS). Six patients with end-stage kidney disease from aHUS received living donor kidney transplantation following induction with anti-thymocyte globulin (3 mg/kg) and maintenance with tacrolimus (level 4–6 ng/dl), enteric-coated mycophenolate sodium (720 mg twice daily), and steroids. Patients were started on ACE Azilsartan (40–80 mg/day). A 2-year follow-up was done. Four were males and two were females. Five patients had genetic mutations in the CFHR 1/CFHR3 , CFH , and DGKE genes identified by whole-exome sequencing: Mutations. Two patients underwent pretransplant native kidney nephrectomy. All patients had a negative CDC and flow cross-match with negative donor-specific antibody. Post transplantation, there was no delayed graft function or vascular thrombosis. Three patients developed altered graft function and underwent graft biopsy: two had acute T-cell-mediated rejection, and one had acute tubular injury. Four patients had a reduction in the number of antihypertensives. One patient developed cytomegalovirus hepatitis. At 2 years post kidney transplantation, zero recurrences, graft losses or vascular thromboses were observed. Eculizumab sparing protocol for kidney transplantation in patients with aHUS and pathogenic genetic mutation of the alternate complement pathway may present options for patients with limited access to eculizumab. Rescue therapy may be needed, and outcomes in patients with antibody-related aHUS need further exploration.