Find in Library

A 46-year-old female who lost her native kidney function due a biopsy proven collapsing type of FSGS, had a living kidney transplant after which, she developed acute renal allograft dysfunction, thrombocytopenia and microangiopathichemolytic anemia five days post-transplant. Renal biopsy revealed acute antibody-mediated rejection (AMR). Genetic studies showed that this patient has a homozygous mutation of complement factor B (CFB) gene and heterozygous variant of C 3 gene consistent with atypical hemolytic uremic syndrome type 4. Intravenous immunoglobulin (IV IG) and plasma exchange did not resolve these abnormalities. Eculizumab and bortezomib, on other hand, were very effective.