Recent advances in understanding and treating immunoglobulin light chain amyloidosis. F1000Res 2018;7
Date
09/20/2018Pubmed ID
30228867Pubmed Central ID
PMC6117860DOI
10.12688/f1000research.15353.1Scopus ID
2-s2.0-85052967237 (requires institutional sign-in at Scopus site) 16 CitationsAbstract
Immunoglobulin (Ig) light chain (AL) amyloidosis is a clonal plasma cell disorder characterized by misfolded Ig light chain deposition in vital organs of the body, resulting in proteotoxicity and organ dysfunction. Owing to its diverse clinical presentations and a tendency to mimic common medical conditions, AL amyloidosis is often diagnosed late and results in dismal outcomes. Early referral to a specialized center with expertise in management of AL amyloidosis is always recommended. The availability of sensitive biomarkers and novel therapies is reforming our approach to how we manage AL amyloidosis. Treatment for patients with AL amyloidosis should be risk-adapted and customized on the basis of individual patient characteristics. In the future, approaches directed at amyloid fibril clearance in combination with agents that target plasma cells will be needed both to eradicate the malignant clone and to establish organ responses.
Author List
Badar T, D'Souza A, Hari PAuthors
Anita D'Souza MD Professor in the Medicine department at Medical College of WisconsinParameswaran Hari MD Adjunct Professor in the Medicine department at Medical College of Wisconsin
MESH terms used to index this publication - Major topics in bold
AnimalsHumans
Immunoglobulin Light Chains
Immunoglobulin Light-chain Amyloidosis
Myeloablative Agonists
