Primary systemic amyloidosis: A brief overview
- PMID: 39672504
- DOI: 10.1016/j.lpm.2024.104267
Primary systemic amyloidosis: A brief overview
Abstract
Primary systemic amyloidosis, or light chain (AL) amyloidosis, is a rare lymphoproliferative disorder in which aberrant light-chain immunoglobulins secreted into the bloodstream aggregate into fibrils and deposit into tissues, causing widespread organ damage and, if not treated, death. This review provides a comprehensive summary of the pathophysiology and manifestations of AL amyloidosis; standard-of-care diagnostic approach; typical treatment regimens; and areas of active investigation.
Keywords: Diagnostic testing; Novel therapeutics; Plasma cell dyscrasias; Primary systemic amyloidosis.
Copyright © 2024 Elsevier Masson SAS. All rights reserved.
Conflict of interest statement
Declaration of interest S.L. is a Consultant and/or Advisor for Adaptive Biotechnologies, Alexion Therapeutics, Bristol-Meyers-Squibb, Caelum Biosciences, Janssen Pharmaceuticals, Karyopharm Therapeutics, Oncopeptides AB, Pfizer, GlaxoSmithKline, Abbvie, and Takeda Pharmaceutical Company; receives research funding from Celgene, Inc., Sanofi, and Zentali; received honoraria from Clinical Care Options and Regeneron Pharmaceuticals; and has Royalties/Patents with Caelum Biosciences. The authors otherwise report no disclosures or conflicts of interest.
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