Orthotopic heart transplant facilitated autologous hematopoietic stem cell transplantation in light-chain amyloidosis

Methodist Debakey Cardiovasc J. Jul-Sep 2012;8(3):17-8. doi: 10.14797/mdcj-8-3-17.

Abstract

Initial manifestations of light-chain amyloidosis (AL) are variable and often result in missed or delayed diagnosis. Survival in AL patients depends mainly on the severity of cardiac involvement. Dominant stage-III cardiac involvement due to primary systemic amyloidosis precludes effective AL treatment and is associated with an average survival of only 3-4 months. The following paper discusses the benefits of orthotopic heart transplantation and autologous hematopoietic stem cell transplantation to improve survival in patients with progressive cardiac AL.

Keywords: AL amyloidosis; autologous hematopoietic stem cell transplantation; light-chain amyloidosis; orthotopic heart transplantation.

Publication types

  • Case Reports

MeSH terms

  • Amyloidosis / metabolism
  • Amyloidosis / surgery*
  • Biopsy
  • Cardiomyopathies / diagnosis
  • Cardiomyopathies / etiology
  • Cardiomyopathies / surgery*
  • Child, Preschool
  • Female
  • Heart Transplantation*
  • Hematopoietic Stem Cell Transplantation*
  • Humans
  • Male
  • Myocardium / pathology
  • Transplantation, Autologous