Bence Jones protein

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"Bence-Jones" redirects here. For Mark Bence-Jones the British author, see Mark Bence-Jones.
A crystal of 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.[1] 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.[2]

The proteins are immunoglobulin light chains (paraproteins) and are produced by neoplastic plasma cells. They can be kappa (most of the time) or lambda.[2] The light chains can be immunoglobulin fragments or single homogeneous immunoglobulins. They are found in urine due to the kidneys' decreased 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.[citation needed] The light chains have historically been detected by heating 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[3][4][5] and AL amyloidosis.[5][6][7][8] 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.

History[edit]

The Bence Jones protein was described by the English physician Henry Bence Jones in 1847 and published in 1848.[9] 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.

References[edit]

  1. ^ Bernier, G. M. & Putnam, F. W. (1963). Nature (London), 200, 223±225.
  2. ^ a b Hoffbrand V, Moss P, Pettit J (2006). Essential Haematology (Essential) (5th ed.). Blackwell Publishing Professional. p. 218. ISBN 1-4051-3649-9. 
  3. ^ Drayson M, Tang LX, Drew R, Mead GP, Carr-Smith H, Bradwell AR (May 2001). "Serum free light-chain measurements for identifying and monitoring patients with nonsecretory multiple myeloma". Blood 97 (9): 2900–2. doi:10.1182/blood.V97.9.2900. PMID 11313287. 
  4. ^ Shaw GR (August 2006). "Nonsecretory plasma cell myeloma--becoming even more rare with serum free light-chain assay: a brief review". Archives of Pathology & Laboratory Medicine 130 (8): 1212–5. doi:10.1043/1543-2165(2006)130[1212:NPCMEM]2.0.CO;2. PMID 16879026. 
  5. ^ a b Katzmann JA, Abraham RS, Dispenzieri A, Lust JA, Kyle RA (May 2005). "Diagnostic performance of quantitative kappa and lambda free light chain assays in clinical practice". Clinical Chemistry 51 (5): 878–81. doi:10.1373/clinchem.2004.046870. PMID 15774572. 
  6. ^ Lachmann HJ, Gallimore R, Gillmore JD, et al. (July 2003). "Outcome in systemic AL amyloidosis in relation to changes in concentration of circulating free immunoglobulin light chains following chemotherapy". British Journal of Haematology 122 (1): 78–84. doi:10.1046/j.1365-2141.2003.04433.x. PMID 12823348. 
  7. ^ Abraham RS, Katzmann JA, Clark RJ, Bradwell AR, Kyle RA, Gertz MA (February 2003). "Quantitative analysis of serum free light chains. A new marker for the diagnostic evaluation of primary systemic amyloidosis". American Journal of Clinical Pathology 119 (2): 274–8. doi:10.1309/LYWM-47K2-L8XY-FFB3. PMID 12579999. 
  8. ^ Akar H, Seldin DC, Magnani B, et al. (December 2005). "Quantitative serum free light chain assay in the diagnostic evaluation of AL amyloidosis". Amyloid 12 (4): 210–5. doi:10.1080/13506120500352339. PMID 16399645. 
  9. ^ Jones HB (1848). "On a new substance occurring in the urine of a patient with mollities ossium". Philosophical Transactions of the Royal Society 138: 55–62. doi:10.1098/rstl.1848.0003.