Saudi Journal of Kidney Diseases and Transplantation (Jan 2017)
Eculizumab experience in an adult patient with atypical hemolytic uremic syndrome
Abstract
Atypical hemolytic-uremic syndrome is a disease characterized by nonimmune hemolytic anemia, thrombocytopenia, and renal failure. In this study, we present a case of a patient with atypical hemolytic-uremic syndrome treated successfully with eculizumab. A 20-year-old female was admitted with clinical signs of atypical hemolytic syndrome. The laboratory findings were as follows: hemoglobin 9.2 g/dL, platelet count 18 × 103/μL, creatinine 4.69 mg/dL, schistocytes were in peripheral blood smear, lactate dehydrogenase 2080 U/L, and emergency plasmapheresis procedure with fresh frozen plasma were initiated. The patient was anuric within 12 h of her admittance. ADAMTS13 activity was normal. Her mother’s cousin developed acute rejection immediately after receiving a renal transplant and died two months later. As she did not respond to the treatment and considering her family history, eculizumab was initiated which resulted in platelet counts starting to rise on day 5, and the patient no longer needed dialysis after 22 days.