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Recent advances in understanding and treating immunoglobulin light chain amyloidosis. F1000Res 2018;7

Date

09/20/2018

Pubmed ID

30228867

Pubmed Central ID

PMC6117860

DOI

10.12688/f1000research.15353.1

Scopus ID

2-s2.0-85052967237   4 Citations

Abstract

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 P

Authors

Anita D'Souza MD Associate Professor in the Medicine department at Medical College of Wisconsin
Parameswaran Hari MD Chief, Professor in the Medicine department at Medical College of Wisconsin




MESH terms used to index this publication - Major topics in bold

Animals
Humans
Immunoglobulin Light Chains
Immunoglobulin Light-chain Amyloidosis
Myeloablative Agonists
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