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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.
The recent introduction of a commercial immunoassay for measurement of free light chains potentially offers an improvement in monitoring disease progression and response to treatment, particularly where the paraprotein is difficult to measure accurately by electrophoresis (for example in light chain myeloma, or where the paraprotein level is ...
Abnormal free light chain production has also been reported to be prognostic of a worse outcome in multiple myeloma [36] [37] [38] and chronic lymphocytic leukaemia. [39] An abnormal light-chain ratio has been defined as a kappa to lambda chain ratio of less than 0.26 or more than 1.65. [32]
Bence Jones proteins are present in 2/3 of multiple myeloma cases. [3] The proteins are immunoglobulin light chains (paraproteins) and are produced by neoplastic plasma cells. They can be kappa (most of the time) or lambda. [3] The light chains can be immunoglobulin fragments or single homogeneous immunoglobulins.
Light chain multiple myeloma is diagnosed in patients who have: a) the criteria for diagnosis of multiple myeloma except having a serum free light chain ratio outside the normal range of 0.26 to 1.65 without evidence of an intact immunoglobulin or free heavy chain; or b) an extreme free light chain ratio, i.e. outside the range of 0.02 to 100 ...
Light chains only (or Bence Jones protein). This may be associated with multiple myeloma or AL amyloidosis. Heavy chains only (also known as "heavy chain disease"); Whole immunoglobulins. If immunoglobulins tend to precipitate within blood vessels with cold, that phenomenon takes the name of cryoglobulinaemia.
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