Use of Eculizumab in atypical hemolytic uremic syndrome after renal transplantation

Claudia Iglesias Teixeira, Roberta Gonçalves Mota, Bruna Gabriela Vieira Afonso, Tatiana Vieira Carneiro, Geraldo Sérgio Gonçalves Meira, Divino Urias Mendonça

To report the use of Eculizumab in atypical hemolytic uremic syndrome (aHUS) after renal transplantation. A 16 year-old patient diagnosed with chronic kidney disease since 2010, due to aHUS, under dialysis. kidney transplantation by deceased donor: February/2012. She showed good clinical evolution until the 14th postoperative day, when he developed a fever, oliguria, worsening of renal function [serum creatinine (CRs): 4.0 mg/dl] and signs of hemolysis [platelets: 110,000 mm3; hemoglobin (Hb): 4.5 g/dL; LDH: 3366 U/L]. Renal biopsy of the graft: thrombotic microangiopathy. Treated with handling blood products (fresh plasma) and plasmapheresis, with improvement of renal function (serum creatinine: 1.46 mg/dl). A week after this complication, fever anemia, signs of hemolysis and ITU restarted then it was handled with ciprofloxacin, methylprednisolone pulse therapy and plasma transfusion (Platelets: 43,000 mm3; Hb: 6.0 mg/dl, reticulocytes; 1.3%, haptoglobin < 5.8 mg/dl, HDL: 1181 U/L). After clinical worsening, it was started a therapy with Eculizumab, 900 mg in every five days for two weeks. There was some progress with good clinical response, characterized by improved renal function, stabilization of aHUS and discharged in five days. Since then, she keeps using Eculizumab 900mg each 15 days with the renal and haematological normalization (Platelets: 160,000 mm3; Hb: 11.4 g/dL). The use of Eculizumab was useful in controlling the ongoing manifestation of aHUS and transplant preservation.