Immunoglobulin light chain amyloidosis

Giampaolo Merlini, Raymond L. Comenzo, David C. Seldin, Ashutosh Wechalekar, Morie A. Gertz

Research output: Contribution to journalArticle

56 Citations (Scopus)

Abstract

Primary light chain amyloidosis is the most common form of systemic amyloidosis and is caused by misfolded light chains that cause proteotoxicity and rapid decline of vital organ function. Early diagnosis is essential in order to deliver effective therapy and prevent irreversible organ damage. Accurate diagnosis requires clinical skills and advanced technologies. The disease can be halted and the function of target organs preserved by the prompt reduction and elimination of the plasma cell clone producing the toxic light chains in the bone marrow. Heart damage is the major determinant of survival, and staging with cardiac biomarkers guides treatment. Two-thirds of patients can benefit from treatment with improved quality of life and extended survival. Future efforts should be directed at early diagnosis, improving the tolerability and efficacy of anti-plasma cell therapy, accelerating recovery of organ function via promoting resorption of amyloid deposits, and developing novel approaches to counter light chain proteotoxicity.

Original languageEnglish
Pages (from-to)143-156
Number of pages14
JournalExpert Review of Hematology
Volume7
Issue number1
DOIs
Publication statusPublished - Feb 2014

Fingerprint

Immunoglobulin Light Chains
Amyloidosis
Light
Plasma Cells
Early Diagnosis
Clinical Competence
Survival
Poisons
Recovery of Function
Amyloid Plaques
Cell- and Tissue-Based Therapy
Therapeutics
Clone Cells
Biomarkers
Bone Marrow
Quality of Life
Technology

Keywords

  • amyloid cardiomyopathy
  • biomarkers
  • novel agents
  • proteotoxicity

ASJC Scopus subject areas

  • Hematology

Cite this

Merlini, G., Comenzo, R. L., Seldin, D. C., Wechalekar, A., & Gertz, M. A. (2014). Immunoglobulin light chain amyloidosis. Expert Review of Hematology, 7(1), 143-156. https://doi.org/10.1586/17474086.2014.858594

Immunoglobulin light chain amyloidosis. / Merlini, Giampaolo; Comenzo, Raymond L.; Seldin, David C.; Wechalekar, Ashutosh; Gertz, Morie A.

In: Expert Review of Hematology, Vol. 7, No. 1, 02.2014, p. 143-156.

Research output: Contribution to journalArticle

Merlini, G, Comenzo, RL, Seldin, DC, Wechalekar, A & Gertz, MA 2014, 'Immunoglobulin light chain amyloidosis', Expert Review of Hematology, vol. 7, no. 1, pp. 143-156. https://doi.org/10.1586/17474086.2014.858594
Merlini, Giampaolo ; Comenzo, Raymond L. ; Seldin, David C. ; Wechalekar, Ashutosh ; Gertz, Morie A. / Immunoglobulin light chain amyloidosis. In: Expert Review of Hematology. 2014 ; Vol. 7, No. 1. pp. 143-156.
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