scholarly journals Recent advances in understanding and treating immunoglobulin light chain amyloidosis

F1000Research ◽  
2018 ◽  
Vol 7 ◽  
pp. 1348 ◽  
Author(s):  
Talha Badar ◽  
Anita D'Souza ◽  
Parameswaran Hari
Keyword(s):  
Light Chain ◽  
Plasma Cells ◽  
Patient Characteristics ◽  
The Body ◽  
Al Amyloidosis ◽  
Specialized Center ◽  
Target Plasma ◽  
Plasma Cell Disorder ◽  
Immunoglobulin Light Chain Amyloidosis ◽  
Light Chain Deposition

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.

scholarly journals Immunoglobulin Light-Chain Amyloidosis: From Basics to New Developments in Diagnosis, Prognosis and Therapy

2016 ◽  
Vol 135 (3) ◽  
pp. 172-190 ◽  
Author(s):  
Eli Muchtar ◽  
Francis K. Buadi ◽  
Angela Dispenzieri ◽  
Morie A. Gertz
Keyword(s):  
Light Chain ◽  
Plasma Cells ◽  
Treatment Options ◽  
Organ Damage ◽  
Major Effect ◽  
Response To Treatment ◽  
Al Amyloidosis ◽  
Prognostic Effect ◽  
Amyloid Light Chain ◽  
Immunoglobulin Light Chain Amyloidosis

Immunoglobulin amyloid light-chain (AL) amyloidosis is the most common form of systemic amyloidosis, where the culprit amyloidogenic protein is immunoglobulin light chains produced by marrow clonal plasma cells. AL amyloidosis is an infrequent disease, and since presentation is variable and often nonspecific, diagnosis is often delayed. This results in cumulative organ damage and has a negative prognostic effect. AL amyloidosis can also be challenging on the diagnostic level, especially when demonstration of Congo red-positive tissue is not readily obtained. Since as many as 31 known amyloidogenic proteins have been identified to date, determination of the amyloid type is required. While several typing methods are available, mass spectrometry has become the gold standard for amyloid typing. Upon confirming the diagnosis of amyloidosis, a pursuit for organ involvement is essential, with a focus on heart involvement, even in the absence of suggestive symptoms for involvement, as this has both prognostic and treatment implications. Details regarding initial treatment options, including stem cell transplantation, are provided in this review. AL amyloidosis management requires a multidisciplinary approach with careful patient monitoring, as organ impairment has a major effect on morbidity and treatment tolerability until a response to treatment is achieved and recovery emerges.

scholarly journals Systemic Light Chain Amyloidosis (Congo Red Inconclusive) with Underlyng Clonal Expansion Not Meeting the Criteria of Light Chain Monoclonal Gammopathy of Renal Significance or Light Chain Myeloma:A Case Report

2020 ◽  
Vol 07 (04) ◽  
pp. 15-19
Author(s):  
Sanjay Kumar ◽  
Keyword(s):  
Light Chain ◽  
Congo Red ◽  
Monoclonal Gammopathy ◽  
Plasma Cells ◽  
Clonal Expansion ◽  
Chronic Diarrhoea ◽  
Al Amyloidosis ◽  
Immunoglobulin Light Chain ◽  
Light Chain Amyloidosis ◽  
Immunoglobulin Light Chain Amyloidosis

Fifty-eight-year-old male admitted for evaluation of nephrotic syndrome and chronic diarrhoea was detected to have Immunoglobulin light chain amyloidosis (AL Amyloidosis) which was congo red inconclusive from renal biopsy. Bone marrow biopsy showed monoclonal plasma cells of 40% and light chain assay showed predominance of immunoglobulin lambda light chain. The diagnosis was neither fitting into the current diagnostic criteria for light chain Monoclonal Gammopathy of Renal Significance (MGRS) nor light chain myeloma. Literature is scarce regarding patients with AL amyloidosis having underlying clonal expansion not meeting the criteria of light chain myeloma or light chain MGRS.

scholarly journals Splenic plasma cells can serve as a source of amyloidogenic light chains

Blood ◽  
2009 ◽  
Vol 113 (7) ◽  
pp. 1501-1503 ◽  
Author(s):  
Alan Solomon ◽  
Sallie D. Macy ◽  
Craig Wooliver ◽  
Deborah T. Weiss ◽  
Per Westermark
Keyword(s):  
Multiple Myeloma ◽  
Bone Marrow ◽  
Monoclonal Antibodies ◽  
Light Chain ◽  
Plasma Cells ◽  
The Body ◽  
Light Chains ◽  
Al Amyloidosis ◽  
Hematopoietic Organ ◽  
Significant Evidence

Abstract Bone marrow-derived clonal plasma cells, as found in systemic amyloidogenic light chain–associated (AL) amyloidosis, are presumed to be the source of light chains that deposit as fibrils in tissues throughout the body. Paradoxically, people with this disorder, in contrast to multiple myeloma, often have a low percentage of such cells, and it is unknown whether this relatively sparse number can synthesize enough amyloidogenic precursor to form the extensive pathology that occurs. To investigate whether another hematopoietic organ, the spleen, also contains monoclonal light chain–producing plasma cells, we have immunostained such tissue from 26 AL patients with the use of antiplasma cell, antifree κ and λ, and anti-VL subgroup-specific monoclonal antibodies (mAbs). In 12 cases, there was statistically significant evidence of a monoclonal population bearing the same κ or λ isotype as that within the bone marrow and identical to the amyloid. Our studies have shown that the spleen may be another source of amyloidogenic light chains.

scholarly journals Beyond the plasma cell: emerging therapies for immunoglobulin light chain amyloidosis

Blood ◽  
2016 ◽  
Vol 127 (19) ◽  
pp. 2275-2280 ◽  
Author(s):  
Brendan M. Weiss ◽  
Sandy W. Wong ◽  
Raymond L. Comenzo
Keyword(s):  
Light Chain ◽  
Plasma Cells ◽  
Organ Damage ◽  
Immunoglobulin Light Chain ◽  
Organ Function ◽  
Fatal Disease ◽  
Mortality And Morbidity ◽  
Amyloid Deposits ◽  
Emerging Therapies ◽  
Immunoglobulin Light Chain Amyloidosis

Abstract Systemic immunoglobulin light chain (LC) amyloidosis (AL) is a potentially fatal disease caused by immunoglobulin LC produced by clonal plasma cells. These LC form both toxic oligomers and amyloid deposits disrupting vital organ function. Despite reduction of LC by chemotherapy, the restoration of organ function is highly variable and often incomplete. Organ damage remains the major source of mortality and morbidity in AL. This review focuses on the challenges posed by emerging therapies that may limit the toxicity of LC and improve organ function by accelerating the resorption of amyloid deposits.

scholarly journals Hereditary systemic immunoglobulin light-chain amyloidosis

Blood ◽  
2015 ◽  
Vol 125 (21) ◽  
pp. 3281-3286 ◽  
Author(s):  
Merrill D. Benson ◽  
Juris J. Liepnieks ◽  
Barbara Kluve-Beckerman
Keyword(s):  
Light Chain ◽  
Plasma Cell Dyscrasia ◽  
Al Amyloidosis ◽  
Constant Region ◽  
Immunoglobulin Light Chain ◽  
Region Gene ◽  
Dna Analyses ◽  
Key Points ◽  
Constant Region Gene ◽  
Immunoglobulin Light Chain Amyloidosis

Key Points Protein and DNA analyses reveal that mutation in the immunoglobulin κ light-chain constant region gene may cause hereditary amyloidosis. Sequencing of immunoglobulin light-chain constant region genes is indicated for patients with AL amyloidosis and no evidence of a plasma cell dyscrasia.

Outcomes of ASCT for cardiac AL-amyloidosis and cardiac light chain deposition disease.

2015 ◽  
Vol 33 (15_suppl) ◽  
pp. 7035-7035
Author(s):  
Al-Ola A. Abdallah ◽  
Shebli Atrash ◽  
Aziz Bakhous ◽  
Daisy Alapat ◽  
Maurizio Zangari
Keyword(s):  
Light Chain ◽  
Al Amyloidosis ◽  
Light Chain Deposition Disease ◽  
Deposition Disease ◽  
Light Chain Deposition

Correlation between serum levels of free light chain and phenotype of plasma cells in bone marrow in primary AL amyloidosis

Amyloid ◽  
2005 ◽  
Vol 12 (1) ◽  
pp. 33-40 ◽  
Author(s):  
Yasuhiro Shimojima ◽  
Masayuki Matsuda ◽  
Takahisa Gono ◽  
Wataru Ishii ◽  
Tomohisa Fushimi ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Light Chain ◽  
Plasma Cells ◽  
Serum Levels ◽  
Free Light Chain ◽  
Al Amyloidosis
Sign in / Sign up

Export Citation Format

Share Document