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Updated: Feb 3, 2026

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Systemic immunoglobulin light chain amyloidosis.

Giampaolo Merlini1,2, Angela Dispenzieri3, Vaishali Sanchorawala4

  • 1Amyloidosis Research and Treatment Center, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy. gmerlini@unipv.it.

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Systemic immunoglobulin light chain amyloidosis is a progressive protein misfolding disease. Early diagnosis and novel therapies improve outcomes, though advanced cardiac involvement remains a challenge.

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Area of Science:

  • Biochemistry
  • Hematology
  • Nephrology

Background:

  • Systemic immunoglobulin light chain amyloidosis involves misfolded proteins causing organ damage.
  • Cardiac and renal damage are predominant, emphasizing the need for early diagnosis.

Purpose of the Study:

  • To review advances in diagnosing and treating systemic immunoglobulin light chain amyloidosis.
  • To highlight the importance of novel biomarkers and imaging in early detection and therapy evaluation.

Main Methods:

  • Review of current diagnostic and therapeutic strategies.
  • Discussion of novel biomarkers, imaging technologies, and emerging therapies.

Main Results:

  • New biomarkers and imaging facilitate earlier diagnosis and assessment of treatment efficacy.
  • Current treatments like proteasome inhibitors and stem cell transplantation improve outcomes for many.
  • Advanced cardiac involvement presents an unmet therapeutic need.

Conclusions:

  • Early diagnosis and risk-stratified treatment are crucial for managing immunoglobulin light chain amyloidosis.
  • Emerging therapies, including passive immunotherapies, offer future promise for improving patient outlooks.