Eculizumab Prevented Recurrence of Atypical Hemolytic Uremic Syndrome in a Kidney Donor after a Third Kidney Transplantation

A man developed atypical Hemolytic Uremic Syndrome (aHUS) 14 months after donating a kidney to his daughter, who also had aHUS. He received kidney transplants 4 and 6 years after his disease debut but aHUS soon recurred in both grafts. Genetic analysis revealed a complement factor H mutation. A third kidney transplantation was performed in 2012. In addition to immunosuppressant, eculizumab (C5 inhibitor), has been given every second week ever since. There has, as yet (Sep 2014) been no rejection or recurrence. However he developed BK nephropathy, which lowered the kidney function permanently. aHUS recurrences in transplantation grafts are major obstacles in patients with factor H mutation, but can be prevented by eculizumab. The optimal dosage needs further studies and evaluation.