Case Report of an ABO-Incompatible Living-Donor Liver Transplant for a Familial Amyloid Polyneuropathy Patient

Exp Clin Transplant. 2015 Oct;13(5):479-81. doi: 10.6002/ect.2014.0076. Epub 2014 Sep 19.

Abstract

Liver transplant is a treatment for familial amyloid polyneuropathy. Few cases of ABO-incompatible living-donor liver transplant for familial amyloid polyneuropathy exist. The outcome of an ABO-incompatible living-donor liver transplant has improved recently, using local infusion therapy and rituximab prophylaxis. Here, we describe a successful ABO-incompatible living-donor liver transplant in a patient with familial amyloid polyneuropathy in whom disease progression ceased at 2 years' follow-up. Additionally, no evidence of acute or chronic rejection, or adverse events of the immunosuppressive therapy, was seen. As a postoperative complication, fatty changes in the grafted liver because of malnutrition or adverse events of corticosteroids were confirmed by a liver biopsy taken early after transplant. The main cause of malnutrition was considered to be gastrointestinal dysfunction caused by familial amyloid polyneuropathy. Therefore, before deterioration of digestive function, liver transplants should be considered for familial amyloid polyneuropathy. This case suggests that an ABO-incompatible living-donor liver transplant may provide greater opportunities for familial amyloid polyneuropathy patients.

Publication types

  • Case Reports

MeSH terms

  • ABO Blood-Group System / immunology*
  • Adult
  • Amyloid Neuropathies, Familial / diagnosis
  • Amyloid Neuropathies, Familial / genetics
  • Amyloid Neuropathies, Familial / immunology
  • Amyloid Neuropathies, Familial / surgery*
  • Biopsy
  • Blood Group Incompatibility / immunology*
  • Graft Rejection / immunology
  • Graft Rejection / prevention & control
  • Graft Survival
  • Histocompatibility*
  • Humans
  • Immunosuppressive Agents / administration & dosage
  • Liver Transplantation / methods*
  • Living Donors*
  • Male
  • Plasma Exchange
  • Rituximab / administration & dosage
  • Time Factors
  • Tomography, X-Ray Computed
  • Treatment Outcome

Substances

  • ABO Blood-Group System
  • Immunosuppressive Agents
  • Rituximab