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Abstract: SA-PO1130

A Unique Immunosuppression Strategy in a Patient with Atypical HUS Undergoing Kidney Transplant

Session Information

Category: Trainee Case Report

  • 1902 Transplantation: Clinical

Authors

  • Zahid, Sohaib, Allegheny Health Network, Pittsburgh, Pennsylvania, United States
  • Krupa, Kristin, AHN, Pittsburgh, Pennsylvania, United States
  • Ranjbar tabar, Kiumars, Allegheny General Hospital, Pittsburgh, Pennsylvania, United States
  • Sureshkumar, Kalathil K., Allegheny Health Network, Pittsburgh, Pennsylvania, United States
  • Hussain, Sabiha Malik, Allegheny Health Network, Pittsburgh, Pennsylvania, United States

Group or Team Name

  • Allegheny Health Network
Introduction

Atypical Hemolytic Uremic Syndrome (aHUS) is a rare disease with estimated incidence of 1-2 per 1,000,000 caused by uncontrolled activation of alternative complement pathway due to genetic mutations mainly in complement inhibitor factors. Herein, we present a patient with atypical HUS who underwent deceased donor kidney transplant (DDKT) and successfully treated with a unique immunosuppressive strategy.

Case Description

A 23 year old female with no significant past medical history had a complicated pregnancy at age 18 with pre-eclampsia requiring emergent cesarean section. Post-partum, she developed acute kidney injury, anemia, thrombocytopenia and elevated LDH with normal ADAMTS-13 levels. Kidney biopsy showed acute thrombotic microangiopathy (TMA). Genetic testing revealed heterozygous missense mutations in complement factor H and complement factor H-related gene 5. She was diagnosed with atypical HUS and initiated on eculizumab but without renal recovery and was maintained on hemodialysis. Five years later patient was called in for DDKT with KDPI of 14%. Her PRA was high at 76%. She received a dose of eculizumab pre-operatively followed induction with by Thymoglobulin and methyl prednisolone. We chose maintenance immunosuppression with belatacept instead of tacrolimus along with mycophenolate mofetil (MMF). Eculizumab was continued. Her creatinine improved to 0.9mg/dl. Two months post-transplant, she developed acute T- cell mediated rejection type 1b successfully treated with methyl prednisolone followed by maintenance steroid therapy and increased dose of MMF. Serum creatinine remains at 1 mg/dl at 1 year follow up.

Discussion

Immunosuppressant management in aHUS patients with high PRA undergoing kidney transplant is challenging. Calcineurin inhibitors and DDKT itself are potential triggers for endothelial damage leading to TMA. Belatacept is a selective T-cell co-stimulation blocker which has not been reported to induce TMA. Our case demonstrates the safety and efficacy of Thymoglobulin induction followed by belatacept/ MMF maintenance along with eculizumab in highly sensitized patient with aHUS.