Significant role of magnetic resonance imaging for the diagnosis and evaluation of cardiac amyloidosis in primary light chain amyloidosis

J Clin Exp Hematop. 2020 Sep 25;60(3):97-102. doi: 10.3960/jslrt.19040. Epub 2020 Aug 8.

Abstract

A 47-year-old male with macroglossia presented with dyspnea on effort and chest pain at rest. Cardiac MRI revealed diffuse global subendocardial late gadolinium enhancement below the left ventricular endocardium and a dark blood pool of intracardiac contrast medium. Tongue biopsy revealed amyloid deposition, which was limited in the myocardium. He was diagnosed with primary light chain amyloidosis. His condition was stage I according to the Mayo Clinic staging system. He underwent autologous peripheral blood stem cell transplantation. On Day 10, he developed chest pain and died suddenly on Day 11. Postmortem examination revealed amyloid deposition throughout the heart.

Keywords: autologous peripheral blood stem cell transplant; cardiac MRI; cardiac amyloidosis; cardiac biomarker; primary AL amyloidosis.

Publication types

  • Case Reports

MeSH terms

  • Amyloidosis / diagnostic imaging*
  • Amyloidosis / therapy
  • Heart / diagnostic imaging*
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged
  • Myocardium / pathology
  • Stem Cell Transplantation