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Light chain deposition disease can affect any organ. [3] Renal involvement is always present and can be identified by microscopic hematuria and proteinuria.Due to the gradual buildup of light chains from plasma filtration, renal function rapidly declines in the majority of patients with LCDD as either acute tubulointerstitial nephritis or rapidly progressing glomerulonephritis.
AL amyloidosis is caused by the deposition of abnormal antibody free light chains. The abnormal light chains are produced by monoclonal plasma cells, and, although AL amyloidosis can occur without diagnosis of another disorder, it is often associated with other plasma cell disorders, such as multiple myeloma and Waldenström's macroglobulinemia. [6]
Light Chain Deposition Disease (LCDD) features deposition of light chains only. This is most commonly associated with κ free light chains. [1] Serum protein electrophoresis or immunofixation is positive in 25-76% of cases, while urine protein electrophoresis or immunofixation is positive in 42-90% of cases. [2]
Amyloid light chains deposition in shoulder joint causes enlarged shoulders, also known as "shoulder pad sign". [18] Amyloid light chain depositions can also cause bilateral symmetric polyarthritis. [18] The deposition of amyloid proteins in the bone marrow without causing plasma cell dyscrasias is called amyloidoma. It is commonly found in ...
Monoclonal free light chains, free heavy chains, or a mixture of these chains can deposit in the kidney and other organs to cause systemic monoclonal immunoglobulin deposition disease; free κ or λ light chains can deposit selectively in the kidney's proximal tubule to cause light chain proximal tubulopathy or in the kidney's distal tubule to ...
These AL light chains are misfolded leading to the disordered deposition of amyloid multimers and fibrils in the glomeruli and blood vessels of the kidney. [3] Other types of amyloidosis related MGRS disorders include heavy chain amyloidosis (deposition of only heavy amyloid chains in the kidney)(AH) and heavy and light chain amyloidosis (AHL ...
The study also included an evaluation of changes in plasma and cerebrospinal fluid biomarkers from baseline to week 76, including P-tau181, P-tau217 and neurofilament light chain, as well as an evaluation of various brain volumes using magnetic resonance imaging (MRI) and amyloid and tau deposition using positron emission tomography (PET) scans ...
AA amyloidosis is a form of amyloidosis, a disease characterized by the abnormal deposition of fibers of insoluble protein in the extracellular space of various tissues and organs. In AA amyloidosis, the deposited protein is serum amyloid A protein (SAA), an acute-phase protein which is normally soluble and whose plasma concentration is highest ...