Bence Jones protein
A Bence Jones protein is a monoclonal globulin protein or immunoglobulin light chain found in the urine, with a molecular weight of 22-24 kDa. Detection of Bence Jones protein may be suggestive of multiple myeloma or Waldenström's macroglobulinemia.
Bence Jones proteins are particularly diagnostic of multiple myeloma in the context of end-organ manifestations such as renal failure, lytic (or "punched out") bone lesions, anemia, or large numbers of plasma cells in the bone marrow of patients. Bence Jones proteins are present in 2/3 of multiple myeloma cases.
The proteins are immunoglobulin light chains (paraproteins) and are produced by neoplastic plasma cells. They can be kappa (most of the time) or lambda. The light chains can be immunoglobulin fragments or single homogeneous immunoglobulins. They are found in urine as a result of decreased kidney filtration capabilities due to renal failure, sometimes induced by hypercalcemia from the calcium released as the bones are destroyed or from the light chains themselves. The light chains have historically been detected by heating a urine specimen (which causes the protein to precipitate) and now by electrophoresis of concentrated urine. More recently, serum free light chain assays have been utilised in a number of published studies which have indicated superiority over the urine tests, particularly for patients producing low levels of monoclonal free light chains, as seen in nonsecretory multiple myeloma and AL amyloidosis. This is primarily because of the re-absorption of free light chains in the kidneys, creating a threshold of light chain production which must be exceeded before measurable quantities overflow into the urine. As such, urinalysis is a fickle witness to changing free light chain production.
The Bence Jones protein was described by the English physician Henry Bence Jones in 1847 and published in 1848. The protein was later sequenced by Frank W. Putnam Sr. at the laboratory of Fred Sanger in Cambridge, who was the first to report the entire sequence.
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