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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.
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 ...
Monoclonal immunoglobulin deposition disease, or MIDD, is a disease characterised by the deposition of monoclonal immunoglobulins on the basement membrane of the kidney. Monoclonal immunoglobulins are produced by monoclonal plasma cells, which are found in a variety of plasma cell dyscrasias .
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]
Once set, light chain class remains fixed for the life of the B lymphocyte. In a healthy individual, the total kappa-to-lambda ratio is roughly 2:1 in serum (measuring intact whole antibodies) or 1:1.5 if measuring free light chains, with a highly divergent ratio indicative of neoplasm. The free light chain ratio ranges from 0.26 to 1.65. [1]
High-resolution electrophoresis and serum and urine immunofixation are recommended to help identify and characterize the monoclonal IgM paraprotein. The light chain of the monoclonal protein is usually the kappa light chain. At times, patients with Waldenström macroglobulinemia may exhibit more than one M protein. Plasma viscosity must be ...
Cases of non-CLL/SLL MBL in which the monoclonal B cells do not express CD5, CD23, CD10, or CD103 but strongly express CD79B and light chain Ig have been tentatively designated as having monoclonal B-cell lymphocytosis of the marginal zone (i.e. CBL-MZ). This term is used because normal marginal zone B-cell lymphocytes express these markers.
Anti-Kappa Light Chain Type Description; Anti-Kappa Light Chain [NH3/41.34] This is a recombinant monoclonal antibody to the kappa light chain. Specifically, [NH3/41.34] binds to IgG antibodies via Western blot. There is no cross reactivity with cynomolgus monkey IgG1 and cynomolgus monkey IgG4, which are recognized by Anti-IgG [NH3/130.5.2]. [23]