Immunoglobulin light chain amyloidosis

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

Research output: Contribution to journalReview article

64 Scopus citations

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 (US)
Pages (from-to)143-156
Number of pages14
JournalExpert Review of Hematology
Volume7
Issue number1
DOIs
StatePublished - Feb 1 2014

Keywords

  • amyloid cardiomyopathy
  • biomarkers
  • novel agents
  • proteotoxicity

ASJC Scopus subject areas

  • Hematology

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  • 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