Autologous Hematopoietic Cell Transplantation for Systemic Light Chain (AL-) Amyloidosis

Heather J. Landau, Morie Gertz, Raymond L. Comenzo

Research output: Chapter in Book/Report/Conference proceedingChapter

1 Scopus citations

Abstract

In patients with light chain (AL-) amyloidosis, the fibril precursors are usually free light chains (FLCs) possessing an abnormal tertiary structure. AL-amyloidosis from symptomatic multiple myeloma is the pattern of organ damage. This chapter describes the epidemiology, pathogenesis, and distinctive clinical aspects of this disease, and offers an approach to the evaluation of patients with AL-amyloidosis. It also summarizes recent clinical research in autologous hematopoietic cell transplantation (HCT) for AL-amyloidosis. The only effective therapeutic approach to systemic AL-amyloidosis remains reduction of amyloid-forming FLCs while providing best supportive care. The FLC assay provides a direct quantitative measure of the fibril-precursor protein and of response when used serially during the course of therapy. The combination of oral cyclophosphamide, thalidomide and dexamethasone (CTD) showed promise in the relapsed setting. Bortezomib is the most active and most useful of the novel agents in the treatment of AL-amyloidosis.

Original languageEnglish (US)
Title of host publicationThomas' Hematopoietic Cell Transplantation
Subtitle of host publicationFifth Edition
PublisherWiley-Blackwell
Pages724-741
Number of pages18
Volume2-2
ISBN (Electronic)9781118416426
ISBN (Print)9781118416006
DOIs
StatePublished - Jan 1 2016

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Keywords

  • Autologous hematopoietic cell transplantation
  • Bortezomib
  • Dexamethasone
  • Fibril-precursor protein
  • Free light chains
  • Oral cyclophosphamide
  • Systemic light chain (AL-)amyloidosis
  • Thalidomide

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Landau, H. J., Gertz, M., & Comenzo, R. L. (2016). Autologous Hematopoietic Cell Transplantation for Systemic Light Chain (AL-) Amyloidosis. In Thomas' Hematopoietic Cell Transplantation: Fifth Edition (Vol. 2-2, pp. 724-741). Wiley-Blackwell. https://doi.org/10.1002/9781118416426.ch60