Early relapse of atypical hemolytic uremic syndrome following ABO-incompatible living–related pediatric kidney re-transplant successfully treated with eculizumab

• Published in: Pediatric nephrology (Berlin, West) West), 2021-10, Vol.36 (10), p.3271-3275
• Main Authors: Stojanovic, Jelena, Adamusiak, Anna, Waters, Aoife, Sebire, Neil J., Kessaris, Nicos, Mamode, Nizam, Marks, Stephen D.
• Format: Article
• Language: English
• Subjects: ABO system; Antibodies, Monoclonal, Humanized; Atypical Hemolytic Uremic Syndrome - drug therapy; Atypical Hemolytic Uremic Syndrome - etiology; Autoantibodies; Blood groups; Brief Report; Child; Child, Preschool; Chronic Disease; Complement factor H; Complement factor I; Development and progression; Female; Health aspects; Hemolytic uremic syndrome; Humans; Kidney; Kidney transplantation; Kidney Transplantation - adverse effects; Kidney transplants; Kidneys; Lymphocytes B; Medicine; Medicine & Public Health; Monoclonal antibodies; Nephrectomy; Nephrology; Patients; Pediatrics; Recurrence; Renal failure; Retrospective Studies; Risk factors; Rituximab; Transplantation; Urology
• ISSN: 0931-041X; 1432-198X
• DOI: 10.1007/s00467-021-05193-7

Background A 3-year-old girl with clinical features of atypical HUS (complement Factor I mutation inherited from an asymptomatic mother and Factor H autoantibodies) was treated with plasma exchange, progressed to kidney failure (KF) aged 4 years, and received an en bloc kidney DCD transplant aged 8 years with primary graft non-function necessitating transplant nephrectomy at the time of transplantation. She subsequently underwent re-transplantation from her father. This is a retrospective study of electronic patient records and medical notes. Case–Diagnosis/Treatment A 9-year-old girl received an ABO-incompatible (ABOi) living–related kidney transplant from her father with recipient and donor blood groups of O and A, respectively, with baseline recipient anti-A titers 1:128 reducing to 1:4 at the time of transplant with B lymphocyte depletion with rituximab and four sessions of immunoadsorption. Six hours post-transplant, she had recurrence of aHUS and received the first dose of eculizumab. She continues on monthly home eculizumab infusions with stable kidney allograft function and negative anti-A titers 7 years post-kidney transplantation. Conclusions This is the first report of a pediatric high-risk ABOi living–related kidney transplantation in whom early relapse of aHUS was successfully treated with eculizumab with good long-term patient and allograft outcome.