Abstract: PUB345
Development of Atypical Hemolytic Uremic Syndrome After Kidney Transplantation in a Patient with C3 Glomerulopathy
Session Information
Category: Transplantation
- 2102 Transplantation: Clinical
Authors
- Maturostrakul, Boonyanuth N., Baystate Medical Center, Springfield, Massachusetts, United States
- Greco, Barbara A., Baystate Medical Center, Springfield, Massachusetts, United States
- Aghi, Anushka, Boston University, Boston, Massachusetts, United States
- Obeidat, Yasin, Baystate Medical Center, Springfield, Massachusetts, United States
- Crisi, Giovanna M., Baystate Medical Center, Springfield, Massachusetts, United States
- Landry, Daniel L., Baystate Medical Center, Springfield, Massachusetts, United States
Introduction
Atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathy (C3GN) are distinct clinical disorders with shared defects in the alternative complement pathway. We present a patient with complement factor mutations and C3GN who developed aHUS following transplantation.
Case Description
A 64 year old female presented for a deceased donor kidney transplant. Native kidney biopsy revealed a C3-dominant mesangioproliferative glomerulonephritis. Genetic testing identified mutations in complement factor I (CFI) and DGKE, and an ADAMTS13 variant of unknown significance. She had no monoclonal gammopathy. C3 level was low prior to transplant. She was treated with mycophenolic acid but was stopped due to side effects including mouth ulcers and urinary tract infections. Following induction with Alemtuzumab and solumedrol, tacrolimus monotherapy was started. Thrombocytopenia and anemia ensued immediately post-transplant and the nadir platelet count and hemoglobin were 9 k/mm3 and 5.9 gm/d by post-operative day (POD) 4. Labs were notable for low C3, elevated lactate dehydrogenase, low haptoglobin, and peripheral blood schistocytes. ADAMTS13 activity level returned adequate at 35.9%. On POD4, Eculizumab was started and immunosuppression was changed from tacrolimus to Belatacept and mycophenolic acid. By POD 11, platelets and haptoglobin normalized. Allograft biopsy at 6 weeks showed thrombotic microangiopathy and mild glomerular mesangial proliferation.
Discussion
C3GN and aHUS are distinct pathologic and clinical entities but share common alternative complement pathway dysregulation. Clinical overlap of C3GN and aHUS has been reported in both native kidneys and following transplantation. Two patients with complement factor H mutations and aHUS in their native kidneys developed C3GN post-transplant.(1) C3GN frequently recurs in the allograft early post-transplant and the mesangial increase may represent recurrence. Repeat biopsy is planned.
Reference:
1. Boyer, O et al. Complement factor H deficiency and post-transplantation glomerulonephritis with isolated C3 deposits. Am. J. Kidney Dis. Off. J. Natl. Kidney Found. 2008; 51: 671–677.