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Light chain deposition disease (LCDD) is a rare blood cell disease which is characterized by deposition of fragments of infection-fighting immunoglobulins, called light chains (LCs), in the body. LCs are normally cleared by the kidneys, but in LCDD, these light chain deposits damage organs and cause disease.
Amyloid light-chain (AL) amyloidosis, also known as primary amyloidosis, is the most common form of systemic amyloidosis. [1] The disease is caused when a person's antibody -producing cells do not function properly and produce abnormal protein fibers made of components of antibodies called light chains .
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]
The most common causes of AA amyloidosis in the West are rheumatoid arthritis, inflammatory bowel disease, psoriasis, and familial Mediterranean fever. [10] People undergoing long-term hemodialysis (14–15 years) can develop amyloidosis from accumulation of light chains of the HLA 1 complex which is normally filtered out by the kidneys. [11]
Each light chain is composed of two tandem immunoglobulin domains: one constant (C L) domain; one variable domain (V L) that is important for binding antigen; The approximate length of a light chain protein is from 211 to 217 amino acids. [3] The constant region determines what class (kappa or lambda) the light chain is. [8]
Free light chains (FLCs) are immunoglobulin light chains that are found in the serum (blood) in an unbound (free) state. In recent decades, measuring the amount of free light chains (FLCs) in the blood has become a practical clinical test. FLC tests can be used to diagnose and monitor diseases like multiple myeloma and amyloidosis.
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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 ...