High-dose melphalan followed by autologous stem cell support in primary systemic AL amyloidosis with multiple organ involvement

Intern Med. 2005 May;44(5):484-9. doi: 10.2169/internalmedicine.44.484.

Abstract

A patient with primary systemic AL amyloidosis achieved partial hematological response after 2 courses of VAD (vincristine, doxorubicin and dexamethasone) and subsequent high-dose melphalan followed by autologous peripheral blood stem cell transplantation despite involvement of multiple organs, including the heart. In this patient natriuretic peptides and free light chains in serum were useful as markers of cardiac involvement and therapeutic effects, respectively. When amyloidosis-related dysfunction is seen in multiple organs, this intensive chemotherapy might be a possible therapeutic option, although several modifications in the regimen and careful management are necessary.

Publication types

  • Case Reports

MeSH terms

  • Amyloid / metabolism
  • Amyloidosis / metabolism
  • Amyloidosis / pathology
  • Amyloidosis / therapy*
  • Antineoplastic Agents, Alkylating / therapeutic use*
  • Biopsy, Needle
  • Bone Marrow / pathology
  • Echocardiography
  • Follow-Up Studies
  • Humans
  • Immunoglobulin Light Chains / blood
  • Male
  • Melphalan / therapeutic use*
  • Middle Aged
  • Myocardium / metabolism
  • Myocardium / pathology
  • Natriuretic Peptides / blood
  • Peripheral Blood Stem Cell Transplantation*
  • Submandibular Gland / metabolism
  • Submandibular Gland / pathology
  • Time Factors
  • Tongue / metabolism
  • Tongue / pathology
  • Transplantation, Autologous

Substances

  • Amyloid
  • Antineoplastic Agents, Alkylating
  • Immunoglobulin Light Chains
  • Natriuretic Peptides
  • Melphalan