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Related Concept Videos

Amyloid Fibrils03:03

Amyloid Fibrils

Amyloid fibrils are aggregates of misfolded proteins.  Under most circumstances, misfolded proteins are either refolded by chaperone proteins or degraded by the proteasome. However, in the case of a mutation or a disease, these proteins can accumulate to form large clusters and often further assemble to form elongated fibers, called fibrils. 
Amyloid deposits were observed as early as 1639 in the liver and the spleen.   In 1854, Rudolph Virchow performed iodine staining, normally used to...
Amyloid Fibrils03:03

Amyloid Fibrils

Amyloid fibrils are aggregates of misfolded proteins.  Under most circumstances, misfolded proteins are either refolded by chaperone proteins or degraded by the proteasome. However, in the case of a mutation or a disease, these proteins can accumulate to form large clusters and often further assemble to form elongated fibers, called fibrils. 
Amyloid deposits were observed as early as 1639 in the liver and the spleen.   In 1854, Rudolph Virchow performed iodine staining, normally used to...
Nephrotic Syndrome I : Introduction01:24

Nephrotic Syndrome I : Introduction

Nephrotic Syndrome is a chronic kidney disorder defined by clinical findings such as severe proteinuria, hypoalbuminemia, hyperlipidemia, and edema. These symptoms result from damage to the glomeruli, the kidney’s filtering units, increasing their permeability to proteins.Definition and Meaning:Proteinuria, defined as the loss of more than 3.5 grams of protein per day in adults, is a crucial feature of nephrotic syndrome. This condition is often accompanied by edema, the accumulation of fluid...
Myasthenia Gravis ll: Pathophysiology01:22

Myasthenia Gravis ll: Pathophysiology

The disease process of myasthenia gravis begins at the neuromuscular junction, where antibodies attack key proteins needed for muscle activation. This immune reaction weakens signal transmission, leading to the characteristic muscle fatigue and weakness that define the condition.Immune-Mediated DamageIn most individuals, antibodies target acetylcholine receptors (AChRs) on the postsynaptic membrane of muscle cells. By blocking acetylcholine binding, these antibodies prevent the nerve signal...
Immunodeficiency Diseases01:25

Immunodeficiency Diseases

Immunodeficiency disorders are conditions in which the immune system's ability to fight infectious disease and cancer is compromised or entirely absent. The immune system comprises a complex network of cells, tissues, and organs that work together to protect the body from potentially harmful invaders. When this system is deficient or not functioning properly, it leaves the body susceptible to infections, diseases, or other complications.
There are three main causes of immunodeficiency disorders...

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Systemic amyloid light-chain amyloidosis beyond ANDROMEDA: Diagnostic challenges and therapeutic updates.

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Screening for Systemic Light-Chain Amyloidosis in Patients Over 60 with λ Monoclonal Gammopathies.

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Related Experiment Video

Updated: Jul 17, 2026

Imaging Amyloid Tissues Stained with Luminescent Conjugated Oligothiophenes by Hyperspectral Confocal Microscopy and Fluorescence Lifetime Imaging
10:04

Imaging Amyloid Tissues Stained with Luminescent Conjugated Oligothiophenes by Hyperspectral Confocal Microscopy and Fluorescence Lifetime Imaging

Published on: October 20, 2017

Systemic immunoglobulin light-chain amyloidosis.

Raymond L Comenzo1

  • 1Department of Medicine, Memorial Sloan-Kettering Cancer Center, New York, NY, USA. comenzor@mskcc.org

Clinical Lymphoma & Myeloma
|January 19, 2007
PubMed
Summary

Systemic immunoglobulin light-chain amyloidosis, caused by abnormal free light chains (FLCs), can be treated by targeting plasma cells. Effective therapies lead to amyloid resorption and improved organ function, offering durable responses.

Area of Science:

  • Hematology
  • Oncology
  • Rare Diseases

Background:

  • Amyloidosis is a rare, fatal condition where amyloid fibrils impair organ function.
  • Systemic immunoglobulin light-chain amyloidosis, the most common type, results from clonal plasma cell production of free light chains (FLCs).
  • FLCs can be quantified using the FLC assay, serving as a therapeutic target.

Purpose of the Study:

  • To review the diagnosis and management of systemic immunoglobulin light-chain amyloidosis.
  • To discuss current and emerging therapeutic strategies for eliminating the underlying plasma cell disorder.
  • To highlight the impact of FLC reduction on amyloid deposition and organ function.

Main Methods:

  • Review of existing literature on amyloidosis diagnosis and treatment.

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Immunoglobulin Gene Sequence Analysis In Chronic Lymphocytic Leukemia: From Patient Material To Sequence Interpretation
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Immunoglobulin Gene Sequence Analysis In Chronic Lymphocytic Leukemia: From Patient Material To Sequence Interpretation

Published on: November 26, 2018

Related Experiment Videos

Last Updated: Jul 17, 2026

Imaging Amyloid Tissues Stained with Luminescent Conjugated Oligothiophenes by Hyperspectral Confocal Microscopy and Fluorescence Lifetime Imaging
10:04

Imaging Amyloid Tissues Stained with Luminescent Conjugated Oligothiophenes by Hyperspectral Confocal Microscopy and Fluorescence Lifetime Imaging

Published on: October 20, 2017

Immunoglobulin Gene Sequence Analysis In Chronic Lymphocytic Leukemia: From Patient Material To Sequence Interpretation
09:02

Immunoglobulin Gene Sequence Analysis In Chronic Lymphocytic Leukemia: From Patient Material To Sequence Interpretation

Published on: November 26, 2018

  • Analysis of therapeutic outcomes for different treatment modalities, including chemotherapy and stem cell transplantation.
  • Evaluation of novel agents and monoclonal antibody approaches in clinical investigation.

    • Main Results:

    • Eliminating pathogenic FLCs can lead to amyloid resorption and organ function improvement.
    • Oral melphalan and dexamethasone is effective for non-transplant-eligible patients but carries myelodysplasia risk.
    • Stem cell transplantation followed by adjuvant therapy shows high response rates and durable hematologic complete responses, often with organ recovery.

    Conclusions:

    • Targeting clonal plasma cells to eliminate FLCs is crucial for managing systemic immunoglobulin light-chain amyloidosis.
    • Established therapies like chemotherapy and stem cell transplantation offer significant benefits.
    • Emerging agents and monoclonal antibodies represent promising future directions for this rare disease.