Light chain (AL) amyloidosis is caused by a small B-cell clone producing light chains that form amyloid deposits and cause organ dysfunction. Chemotherapy aims at suppressing the production of the toxic light chain (LC) and restore organ function ...
G. Palladini +24 more
semanticscholar +1 more source
Duodenal biopsy: an unexpected confirmatory test for a patient with al-amyloidosis
SSystemic AL- amyloidosis is a disorder of protein folding in which there is extra-cellular accumulation as β pleated fibrillar deposits of monoclonal immunoglobulin light chain fragments. AL- amyloidosis is a rare clinical entity.
S. Pirasath +4 more
doaj +1 more source
Amyloid light-chain (AL) amyloidosis after kidney transplantation is a rare disease in the world, and only one case of systemic AL amyloidosis after kidney transplantation is reported in Korea.
Sung Hyun Park +8 more
doaj +1 more source
Case Report: Diagnosis of Dual-Biopsy Negative Severe Cardiac Amyloidosis [PDF]
INTRODUCTION Amyloidosis is a rare disorder that involves the deposition of misfolded protein in extracellular tissue. Disease manifestations vary depending on the affected organs.
Kasprowicz, MD, Eric, Lohr, MD, Kristin
core +2 more sources
Incidence and clinical outcome of renal amyloidosis: A retrospective study
The kidneys are affected in almost all patients with amyloid A in secondary amyloidosis (AA) amyloidosis but less frequently in immunoglobulin light chains in primary systemic amyloidosis (AL) amyloidosis.
Emad Abdallah, Emam Waked
doaj +1 more source
Daratumumab plus CyBorD for patients with newly diagnosed light chain (AL) amyloidosis
Primary systemic immunoglobulin light chain (AL) amyloidosis is caused by a plasma cell clone of, usually low, malignant potential that expresses CD38 molecules on their surface.
Foteini Theodorakakou +2 more
doaj +1 more source
Alterations of the gut microbiota in patients with immunoglobulin light chain amyloidosis
BackgroundEmerging evidence revealed that gut microbial dysbiosis is implicated in the development of plasma cell dyscrasias and amyloid deposition diseases, but no data are available on the relationship between gut microbiota and immunoglobulin light ...
Jipeng Yan +15 more
doaj +1 more source
Uncovering the Mechanism of Aggregation of Human Transthyretin. [PDF]
The tetrameric thyroxine transport protein transthyretin (TTR) forms amyloid fibrils upon dissociation and monomer unfolding. The aggregation of transthyretin has been reported as the cause of the life-threatening transthyretin amyloidosis.
Cascio, Duilio +9 more
core +2 more sources
Human central nervous system (CNS) ApoE isoforms are increased by age, differentially altered by amyloidosis, and relative amounts reversed in the CNS compared with plasma [PDF]
The risk of Alzheimer's disease (AD) is highly dependent on apolipoprotein-E (apoE) genotype. The reasons for apoE isoform-selective risk are uncertain; however, both the amounts and structure of human apoE isoforms have been hypothesized to lead to ...
Baker-Nigh, Alaina T +11 more
core +3 more sources
A bibliometric analysis of light chain amyloidosis from 2005 to 2024: research trends and hot spots
BackgroundLight chain (AL) amyloidosis stands as the most prevalent subtype of systemic amyloidosis, encompassing a group of rare diseases. Here, we evaluated the scientific landscape of AL amyloidosis to investigate research trends and identify hotspots
Xiangdong Liu +19 more
doaj +1 more source

