Bence Jones protein

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.

A crystal of Bence Jones protein.

Bence Jones proteins are particularly diagnostic of multiple myeloma in the context of target organ manifestations such as kidney 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 as a result of decreased kidney filtration capabilities due to kidney failure, sometimes induced by hypercalcemia from the calcium released as the bones are destroyed ,dehydration due to polyurea, amyloidosis 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.[3] 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[4][5][6] and AL amyloidosis.[6][7][8][9]

History

The Bence Jones protein was described by the English physician Henry Bence Jones in 1847 and published in 1848.[10]

References
  1. Bernier, G. M. & Putnam, F. W. (1963). Nature (London), 200, 223±225.
  2. Hoffbrand V, Moss P, Pettit J (2006). Essential Haematology (Essential) (5th ed.). Blackwell Publishing Professional. p. 218. ISBN 978-1-4051-3649-5.
  3. "Bence Jones Protein Test". TheFreeDictionary.com.
  4. 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.
  5. 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:10.5858/2006-130-1212-NPCMEM. PMID 16879026.
  6. 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.
  7. 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. S2CID 23475887.
  8. 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.
  9. 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. S2CID 7839338.
  10. 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. S2CID 186208792.
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