Abstract: SA-PO1139
De Novo Transthyretin Amyloidosis After Domino Liver Transplant Causing Kidney Graft Failure: A Case Report
Session Information
- Transplant Trainee Case Reports
November 09, 2019 | Location: Exhibit Hall, Walter E. Washington Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Trainee Case Report
- 1902 Transplantation: Clinical
Authors
- Reis, Fábio A., University of Sao Paulo (USP), Sao Paulo, Brazil
- Santa Catharina, Guilherme P., University of Sao Paulo (USP), Sao Paulo, Brazil
- Smolentzov, Igor, University of Sao Paulo (USP), Sao Paulo, Brazil
- De paula, Flavio, University of Sao Paulo (USP), Sao Paulo, Brazil
- David-Neto, Elias, University of Sao Paulo (USP), Sao Paulo, Brazil
- Reusing, Jose Otto, University of Sao Paulo (USP), Sao Paulo, Brazil
Introduction
Domino Liver Transplant (DLT) refers to a sequential transplant (Tx) where a recipient receives a liver from a donor who has usually Familial Amyloid Polyneuropathy (FAP). DLT increases the chance of getting a liver but poses the risk of the recipient manifesting amyloidosis years later. The current case describes a patient who received a kidney Tx 13 years after a DLT and developed de novo systemic amyloidosis with neuropathy.
Case Description
Male 69 yo, diagnosed with Hepatitis C and Hepatocellular Carcinoma, underwent a DLT transplant with a liver from a FAP donor. After 13 years of Tx he developed ESRD due to Cyclosporine toxicity and recurrent pyelonephritis. One year later he underwent a deceased donor kidney Tx under Basiliximab, Mycophenolate and Tacrolimus immunosuppression. Three years later, he presented progressive lower limbs paresthesia and weakness (electroneuromyography confirmed polineuropathy); anasarca, dyspnea and bilateral pleural effusion (echocardiography showed new diastolic dysfunction and myocardial hypertrophy) and progressive symptomatic hypotension episodes. Because of non-proteinuric subacute kidney graft dysfunction and dyspnea he was hospitalized and initiated hemodialysis. Kidney graft biopsy demonstrated acute tubular necrosis (ATN), light glomerulitis, C4d and Congo Red negative, and no signs of Tacrolimus toxicity. On the other hand, sural nerve biopsy stained Congo Red positive and confirmed Amyloid Neuropathy. Graft failure was attributed to ischemic injury, due to arterial hypotension secondary to autonomic neuropathy. Other features as neurogenic bladder with post-void retention and new myocardial hypertrophy without arterial hypertension were also attributed to amyloidosis. Dialysis ultrafiltration was progressively hindered by untreatable hypotension and he died 2 months later.
Discussion
This case illustrates the occurrence of Transthyretin Amyloidosis with severe manifestations in a recipient of DLT. We point out the differential diagnosis of kidney graft failure, here described as ATN as an indirect consequence of Amyloidosis.