Abstract: SA-PO018

Role of Liver Transplantation in the Treatment of Fibrinogen Aa-Chain Amyloidosis

Session Information

Category: Transplantation

  • 1802 Transplantation: Clinical

Authors

  • Stangou, Arie, Queen Elizabeth Hospital, Birmingham, United Kingdom
  • Zeldenrust, Steven R., Mayo Clinic, Rochester, Minnesota, United States
  • Leung, Nelson, Mayo Clinic, Rochester, Minnesota, United States
  • Pinney, Jennifer H., Department of Renal Medicine, University Hospital Birmingham,, Worcestershire, United Kingdom
  • Drosou, Maria Eleni, Mayo Clinic, Rochester, Minnesota, United States
  • Schwab, Thomas R., Mayo Clinic Rochester, Rochester, Minnesota, United States
  • Fix, Oren, Swedish Medical Center, Seattle, Washington, United States
  • Wiesner, Russell Harold, Mayo clinic, Rochester, Minnesota, United States
  • Benson, Merrill D., Indiana University School of Medicine, Indianapolis, Indiana, United States
Background

Variants of fibrinogen Aα-chain (AFib) cause the commonest type of hereditary amyloid nephropathy in Europe and probably the US. Fibrinogen production is exclusively hepatic and kidney transplants often fail within 1-5 years with recurrent amyloidosis. The addition of liver transplantation (LT) offers curative potential. We present the long-term experience of the first 15 AFib patients who received liver grafts in our Institutions in the UK and US, and discuss the merits and scope for optimal utilisation of this valuable resource.

Methods

Ten British and 5 US patients with AFib E526V and amyloid nephropathy were listed for transplantation between 1995-2017. Dominant features were proteinuria (66%) median 7.37 (2.5-9.8) g/D and kidney impairment, median GFR 25 (0-104) ml/min . Extra-renal amyloid manifestations included cardiac (40%) and gastrointestinal (66.6%) autonomic neuropathy and clinically significant splenic amyloidosis (27%). Thirteen patients, median age 59 (49-63), 9 male, received liver-kidney transplant (LKT); two females aged 54 and 58, kidney disease stage 1 and 3 respectively, received isolated liver grafts (LT).

Results

At median posttransplant fol-up 133 months (12-277), there is no amyloid progression and cumulative patient survival in the entire cohort is 80%. LKT patient survival is 76.9%, kidney/ liver graft 92.3%, posttransplant GFR 46 (41-78) ml/min. Three adverse outcomes due to biliary or vascular complications occurred early postoperatively in the subgroup of 9 patients who received LKT after long-term haemodialysis (survival only 66.7%). In contrast, in those receiving pre-emptive LKT or LT alone patient survival was 100%. Both recipients of isolated LT maintain normal liver and stable renal function with diminishing proteinuria at 12 and 95 months.

Conclusion

The therapeutic potential of liver transplantation in fibrinogen amyloidosis is truly curative. Best transplant benefit from the addition of liver to kidney grafts was achieved in low risk pre-dialysis patients. Timely intervention in the pre-dialysis setting utilising liver transplantation alone is rational, feasible and effective. We support consideration of this approach to halt disease progression and prevent haemodialysis.