Light Chain Stabilization: A Therapeutic Approach to Ameliorate AL Amyloidosis
Non-native immunoglobulin light chain conformations, including aggregates, appear to cause light chain amyloidosis pathology. Despite significant progress in pharmacological eradication of the neoplastic plasma cells that secrete these light chains, in ...
Gareth J. Morgan +2 more
doaj +1 more source
Natural history and outcome in systemic AA amyloidosis [PDF]
BACKGROUND:Deposition of amyloid fibrils derived from circulating acute-phase reactant serum amyloid A protein (SAA) causes systemic AA amyloidosis, a serious complication of many chronic inflammatory disorders.
Gallimore, JR +6 more
core +1 more source
Cutaneous light chain amyloidosis with multiple myeloma: A concise review
Objective/Background: Cutaneous immunoglobulin (Ig) amyloid light-chain (AL) amyloidosis associated with overt multiple myeloma (MM) is rare and optimal treatment is not well defined.
Mirela Andrei, Jen Chin Wang
doaj +1 more source
Pseudoxanthoma Elasticum and Light-Chain Amyloidosis [PDF]
Pseudoxanthoma elasticum is a heritable disorder of connective tissue characterized by cutaneous, vascular and ocular changes that result from the accumulation of fragmented elastic fibres. Even though the etiopathogenesis is not still completely understood, in recent years in literature some Authors have considered pseudoxanthoma elasticum as a ...
M. Carlesimo +8 more
openaire +2 more sources
?2-Microglobulin Amyloid Fibril-Induced Membrane Disruption Is Enhanced by Endosomal Lipids and Acidic pH [PDF]
Although the molecular mechanisms underlying the pathology of amyloidoses are not well understood, the interaction between amyloid proteins and cell membranes is thought to play a role in several amyloid diseases. Amyloid fibrils of ?2-microglobulin (?2m)
A Halle +98 more
core +7 more sources
Amyloidosis: a case series and review of the literature
Background Systemic amyloidosis is group of disorders characterized by the accumulation of insoluble proteins in tissues. The most common form of systemic amyloidosis is light chain amyloidosis, which results from the accumulation of misfolded ...
Justin B. Senecal +6 more
doaj +1 more source
Concurrent nephrotic syndrome and acute renal failure caused by chronic lymphocytic leukemia (CLL): a case report and literature review [PDF]
Kidney injury associated with lymphocytic leukemia (CLL) is typically caused by direct tumor infiltration which occasionally results in acute renal failure. Glomerular involvement presenting as proteinuria or even nephrotic syndrome is exceptionally rare.
Xianrui Dou +6 more
core +2 more sources
Predicting survival in light chain amyloidosis
In this issue of the Journal, Dittrich and coworkers report their systematic analysis of the performance of the three currently used staging systems for AL amyloidosis in a large population of 1,224 patients.[1][1] Their aim was to establish whether one of the available models had ...
Giovanni Palladini +2 more
doaj +4 more sources
Biomarkers in Immunoglobulin Light Chain Amyloidosis
Immunoglobulin light chain amyloidosis (AL amyloidosis - ALA) is a monoclonal gammopathy characterized by presence of aberrant plasma cells producing amyloidogenic immunoglobulin light chains. This leads to formation of amyloid fibrils in various organs and tissues, mainly in heart and kidney, and causes their dysfunction.
Kufova, Z. +17 more
openaire +4 more sources
Misdiagnosis of hereditary amyloidosis as AL (Primary) amyloidosis [PDF]
Background: Hereditary, autosomal dominant amyloidosis, caused by mutations in the genes encoding transthyretin, fibrinogen A -chain, lysozyme, or apolipoprotein A-I, is thought to be extremely rare and is not routinely included in the differential ...
Booth, D.R. +7 more
core

