Immunoglobulin light chain systemic amyloidosis

Angela Dispenzieri, Giampaolo Merlini

Research output: Chapter in Book/Report/Conference proceedingChapter

12 Scopus citations

Abstract

Immunoglobulin light chain amyloidosis (AL) is a rare, complex disease caused by misfolded free light chains produced by a usually small, indolent plasma cell clone. Effective treatments exist that can alter the natural history, provided that they are started before irreversible organ damage has occurred. The cornerstones of the management of AL amyloidosis are early diagnosis, accurate typing, appropriate risk-adapted therapy, tight follow-up, and effective supportive treatment. The suppression of the amyloidogenic light chains using the cardiac biomarkers as guide to choose chemotherapy is still the mainstay of therapy. There are exciting possibilities ahead, including the study of oral proteasome inhibitors, antibodies directed at plasma cell clone, and finally antibodies attacking the amyloid deposits are entering the clinic, offering unprecedented opportunities for radically improving the care of this disease.

Original languageEnglish (US)
Title of host publicationCancer Treatment and Research
PublisherKluwer Academic Publishers
Pages273-318
Number of pages46
Volume169
DOIs
StatePublished - 2016

Publication series

NameCancer Treatment and Research
Volume169
ISSN (Print)09273042

Keywords

  • Biomarkers
  • Cardiac amyloidosis
  • Chemotherapy
  • Immunoglobulin light chain amyloidosis
  • Immunotherapy

ASJC Scopus subject areas

  • Medicine(all)
  • Oncology
  • Cancer Research

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  • Cite this

    Dispenzieri, A., & Merlini, G. (2016). Immunoglobulin light chain systemic amyloidosis. In Cancer Treatment and Research (Vol. 169, pp. 273-318). (Cancer Treatment and Research; Vol. 169). Kluwer Academic Publishers. https://doi.org/10.1007/978-3-319-40320-5_15