Improvement of cardiac diastolic function and prognosis after autologous peripheral blood stem cell transplantation in AL cardiac amyloidosis

Intern Med. 2007;46(20):1705-10. doi: 10.2169/internalmedicine.46.0142. Epub 2007 Oct 15.

Abstract

AL amyloidosis is a disease in which immunoglobulin L chain is deposited in multiple organs, and the prognosis of cardiac amyloidosis is extremely poor. Although several treatments based on that for multiple myeloma, have been performed, there is no clear evidence that cardiac function is improved. We report a case of AL cardiac amyloidosis with moderate cardiac dysfunction for which we performed autologous peripheral blood stem cell transplantation (auto-PBSCT) in combination with high-dose melphalan therapy. This treatment resulted in significant improvement in cardiac function and good prognosis for about 3.5 years after the diagnosis. Therefore, auto-PBSCT is a possible option as up-front therapy for AL cardiac amyloidosis.

Publication types

  • Case Reports

MeSH terms

  • Amyloidosis / physiopathology
  • Amyloidosis / therapy*
  • Cardiomyopathies / physiopathology
  • Cardiomyopathies / therapy*
  • Combined Modality Therapy
  • Female
  • Heart / physiopathology
  • Humans
  • Melphalan / administration & dosage
  • Middle Aged
  • Peripheral Blood Stem Cell Transplantation*
  • Prognosis

Substances

  • Melphalan