Immunoglobulin light chain systemic amyloidosis

Angela Dispenzieri, Giampaolo Merlini

Research output: Chapter in Book/Report/Conference proceedingChapter

10 Citations (Scopus)

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
Title of host publicationCancer Treatment and Research
PublisherKluwer Academic Publishers
Pages273-318
Number of pages46
Volume169
DOIs
Publication statusPublished - 2016

Publication series

NameCancer Treatment and Research
Volume169
ISSN (Print)09273042

Fingerprint

Immunoglobulin Light Chains
Amyloidosis
Plasma Cells
Clone Cells
Light
Proteasome Inhibitors
Antibodies
Amyloid Plaques
Therapeutics
Rare Diseases
Natural History
Early Diagnosis
Biomarkers
Drug Therapy

Keywords

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

ASJC Scopus subject areas

  • Medicine(all)
  • Oncology
  • Cancer Research

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

Immunoglobulin light chain systemic amyloidosis. / Dispenzieri, Angela; Merlini, Giampaolo.

Cancer Treatment and Research. Vol. 169 Kluwer Academic Publishers, 2016. p. 273-318 (Cancer Treatment and Research; Vol. 169).

Research output: Chapter in Book/Report/Conference proceedingChapter

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