Immunoglobulin light chain amyloidosis diagnosis and treatment algorithm 2021 [PDF]
Blood Cancer Journal, 2021Immunoglobulin light chain amyloidosis (AL) commonly presents with nephrotic range proteinuria, heart failure with preserved ejection fraction, nondiabetic peripheral neuropathy, unexplained hepatomegaly or diarrhea, and should be considered in patients ...
M. Hasib Sidiqi, Morie A. Gertz
doaj +3 more sources
Daratumumab-Based Treatment for Immunoglobulin Light-Chain Amyloidosis. [PDF]
New England Journal of Medicine, 2021BACKGROUND Systemic immunoglobulin light-chain (AL) amyloidosis is characterized by deposition of amyloid fibrils of light chains produced by clonal CD38+ plasma cells. Daratumumab, a human CD38-targeting antibody, may improve outcomes for this disease.
E. Kastritis+49 more
semanticscholar +14 more sources
Immunoglobulin light chain amyloid aggregation. [PDF]
Chem Commun (Camb), 2018Light chain (AL) amyloidosis is a devastating, complex, and incurable protein misfolding disease. It is characterized by an abnormal proliferation of plasma cells (fully differentiated B cells) producing an excess of monoclonal immunoglobulin light ...
Blancas-Mejia LM+8 more
europepmc +6 more sources
Systemic immunoglobulin light chain amyloidosis [PDF]
Nature Reviews Disease Primers, 2018Systemic immunoglobulin light chain amyloidosis is a protein misfolding disease caused by the conversion of immunoglobulin light chains from their soluble functional states into highly organized amyloid fibrillar aggregates that lead to organ dysfunction.
G. Merlini+6 more
semanticscholar +7 more sources
Differences in immunoglobulin light chain species found in urinary exosomes in light chain amyloidosis (Al). [PDF]
PLoS ONE, 2012Renal involvement is a frequent consequence of plasma cell dyscrasias. The most common entities are light chain amyloidosis, monoclonal immunoglobulin deposition disease and myeloma cast nephropathy.
Marina Ramirez-Alvarado+7 more
doaj +12 more sources
Genetic pathogenesis of immunoglobulin light chain amyloidosis: basic characteristics and clinical applications [PDF]
Experimental Hematology & Oncology, 2021Immunoglobulin light chain amyloidosis (AL) is an indolent plasma cell disorder characterized by free immunoglobulin light chain (FLC) misfolding and amyloid fibril deposition.
Linchun Xu, Yongzhong Su
doaj +2 more sources
Germline polymorphisms and alternative splicing of human immunoglobulin light chain genes [PDF]
iScience, 2021Summary: Inference of germline polymorphisms in immunoglobulin genes from B cell receptor repertoires is complicated by somatic hypermutations, sequencing/PCR errors, and by varying length of reference alleles.
Ivana Mikocziova+5 more
doaj +2 more sources
Localized Immunoglobulin Light-Chain Amyloidosis of the Ulnar Nerve [PDF]
Case Reports in Neurology, 2021Amyloidosis is a disorder caused by extracellular tissue deposition of insoluble fibrils. Amyloidosis can be divided into systemic or localized disease. Primary systemic amyloidosis is a multisystem disease caused by the deposition of amyloid in various ...
Shinsuke Morisaki+5 more
doaj +2 more sources
AL amyloidosis with non-amyloid forming monoclonal immunoglobulin deposition; a case mimicking AHL amyloidosis [PDF]
BMC Nephrology, 2018Background Immunoglobulin heavy-and-light-chain amyloidosis (AHL amyloidosis) is a newly established disease entity where both the immunoglobulin heavy-chain and light-chain compose amyloid fibrils. The immunoglobulins responsible for the amyloid fibrils
Shun Manabe+6 more
doaj +3 more sources
Inhibition by small-molecule ligands of formation of amyloid fibrils of an immunoglobulin light chain variable domain [PDF]
eLife, 2015Overproduction of immunoglobulin light chains leads to systemic amyloidosis, a lethal disease characterized by the formation of amyloid fibrils in patients' tissues. Excess light chains are in equilibrium between dimers and less stable monomers which can
B. Brumshtein+7 more
semanticscholar +3 more sources