[Heart transplantation in AL amyloidosis]

Lakartidningen. 2018 Mar 26:115:EY3F.
[Article in Swedish]

Abstract

Amyloidosis is a disease complex characterized by the deposition of protein fibrils in various tissues, which leads to structural and functional derangement of the affected organ. There are different types of amyloidosis categorized on the basis of the type of protein fibrils deposited. Cardiac involvement has been predominantly noted in amyloid light chain (AL) amyloidosis and is the major prognostic determinant and influences the therapeutic strategy. In AL amyloidosis, heart transplantation is generally not recommended because of a high risk of recurrence in the transplanted heart and poor survival rate. However, a favourable outcome can be achieved if heart transplantation is followed by an autologous stem cell transplantation (ASCT). We describe our experience from the two first patients with AL amyloidosis treated with heart transplantation and subsequent ASCT at Sahlgrenska University Hospital.

Publication types

  • Case Reports

MeSH terms

  • Female
  • Heart Failure / etiology
  • Heart Failure / surgery
  • Heart Transplantation*
  • Humans
  • Immunoglobulin Light-chain Amyloidosis / complications
  • Immunoglobulin Light-chain Amyloidosis / pathology
  • Immunoglobulin Light-chain Amyloidosis / surgery*
  • Immunoglobulin lambda-Chains / blood
  • Male
  • Middle Aged
  • Stem Cell Transplantation*
  • Transplantation, Autologous

Substances

  • Immunoglobulin lambda-Chains