Diagnosis and management of systemic light chain AL amyloidosis.

Divaya Bhutani, Suzanne Lentzsch
Author Information
  1. Divaya Bhutani: Department of Internal Medicine, Division of Hematology/Oncology, Columbia University Medical Center, New York, NY, United States of America. Electronic address: db3203@cumc.columbia.edu.
  2. Suzanne Lentzsch: Department of Internal Medicine, Division of Hematology/Oncology, Columbia University Medical Center, New York, NY, United States of America.

Abstract

AL amyloidosis is a plasma cell disorder leading to the production and extracellular deposition of abnormal immunoglobulin light chains called amyloid. The pathogenesis of the disorder is driven by an abnormal plasma cell clone producing excessive monoclonal immunoglobulin light chains that undergo deposition in various organs of the body such as the heart, kidney, and gastrointestinal tract. The outcome of the disease remains poor with significant morbidity and mortality associated with organ dysfunction. In this review, we describe the current standard diagnostic features, prognosis, and current treatment paradigm of the disease.

Keywords

MeSH Term

Amyloid
Animals
Antibodies, Monoclonal, Humanized
Biomarkers
Cardiomyopathies
Disease Progression
Humans
Immunoglobulin Light Chains
Immunoglobulin Light-chain Amyloidosis
Plaque, Amyloid
Plasma Cells
Predictive Value of Tests
Proteasome Inhibitors
Stem Cell Transplantation
Treatment Outcome

Chemicals

Amyloid
Antibodies, Monoclonal, Humanized
Biomarkers
Immunoglobulin Light Chains
Proteasome Inhibitors

Word Cloud

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