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Abstract: SA-PO1119

An Interesting Case of Anti-HLA-C Antibody-Mediated Acute Humoral Rejection and Fabry-Like Zebra Bodies in a Renal Transplant Recipient

Session Information

Category: Trainee Case Report

  • 1902 Transplantation: Clinical

Authors

  • Abuzeineh, Mohammad W.K, University Of Missouri, Columbia, Missouri, United States
  • Ziadeh, Ahmad Jihad, University Of Missouri, Columbia, Missouri, United States
  • Yerram, Preethi, University Of Missouri, Columbia, Missouri, United States
Introduction

Detection of Donor Specific Anti-bodies (DSA) is essential in diagnosing Antibody-mediated renal allograft rejection (AMR). HLA-C antibodies testing is not part of routine DSA pre-transplant evaluation, but they have been reported to cause AMR. We present a case of AMR secondary to DSA against HLA-C, and incidental finding of ultrastructural zebra-bodies under Electron Microscopy (EM), raising suspicion for undiagnosed Fabry’s disease, eventually determined to be of uncertain significance.

Case Description

A 39-year old African American male with ESRD due to hypertensive nephropathy underwent 2A/2B/2DR mismatched cadaveric transplant in 2014. Post-transplant Creatinine (Cr) was 1.4-1.6 mg/dl. Four years later, Cr was found to be elevated at 2.16 mg/dl. Allograft biopsy showed early chronic transplant glomerulopathy without evidence of acute cellular or humoral rejection. EM showed ultrastructural zebra-patterned lipid inclusions in podocytes (Fig 1), suspicious for donor-derived Fabry’s disease, which turned out to be of unclear significance. Cr worsened and he developed nephrotic range proteinuria and hematuria. Repeat biopsy showed capillaritis and arteritis with focal fibrinoid necrosis (Fig 2), with weak and focal C4d deposits on IF. DSA were positive to Cw7 and Cw2. He was treated for acute AMR with pulse steroids, IV immunoglobulin, plasmapheresis and rituximab but his renal function worsened and he became dialysis-dependent.

Discussion

This case report emphasizes the role of HLA-C antibodies in causing AMR, and demonstrates the need for their recognition in the pre- and post-transplant period. In addition, the incidental presence of zebra-patterned lipid inclusions in podocytes in transplant renal biopsy doesn't necessarily indicate Fabry’s disease.