The bence jones protein urine test is one of many tests that doctors can do if they suspect that someone has multiple myeloma, which is a form of blood cancer. Electrophoresis separates proteins based on their physical properties, and the. The specific immunoglobulin component is unassociated kappa or lambda light chains. Addition of a strong mineral acid to the urine caused precipitation of the protein, which dissolved when the mixture was. The urine mspike is used for diagnosis and response determination in multiple. Bence jones proteins are molecules of about 40,000 mol wt present in the urine of many patients with plasma cell malignancy. Bence jones proteins and light chains are considered as a single population and the variable and constant regions. Detect and type bence jones proteins monoclonal kappa andor lambda free light chains or any other monoclonal full immunoglobulin.
Bence jones proteins are particularly diagnostic of multiple myeloma in the context of endorgan manifestations such as renal failure, lytic bone lesions, anemia, or large numbers of plasma cells in the bone marrow of patients. Detection of bencejones protein in practice rbeetham from the department of clinical biochemistry, frenchay hospital, north bristol hospitals nhs trust, bristol bs16 1le, uk introduction the monoclonal gammopathies1are characterized by the presence in serum andor urine of a monoclonal, intact immunoglobulin or frag. Lars b nielsen, simple method for quantification of bence jones proteins, clinical chemistry, volume 48, issue 12, 1 december 2002, pages 22022207. Bencejones proteins are a part of regular antibodies called light chains. The presence of bence jones protein in the urine, anemia, and increased m protein on serum protein electrophoresis point strongly to the diagnosis of multiple myeloma.
Simple method for quantification of bence jones proteins morten salomo. An antiserum prepared against a lambdabence jones protein from a patient sut who had multiple myeloma and amyloidosis had specificity for lambdalight chains of the chemically defined variable v region lambdachain subgroup lambda vi. Korngold and lapiri characterised two types of bence jones protein subsequently designated. The proteins excreted in the urine of patients suffering from multiple myeloma were originally studied by dr. Proteins with unusual thermosolubility found in the urine of patients with multiple myeloma, consisting of monoclonal immunoglobulin light chains.
Bence jones protein and its renal metabolism bence jones protein derives its name from the studies of dr henry bence jones and others in 184516but it was not until the early 1960s that it was established as being a monoclonal light chain. If only bencejones protein is present, the test is clearcut. Bencejones protein definition is a polypeptide composed of one or two antibody light chains that is found especially in the urine of persons affected with multiple myeloma. Bencejones proteins are urinary free light chains detected by urinary protein electrophoresis and immunofixation. Bence jones protein an overview sciencedirect topics. More than a century ago bence jones described a remarkable protein in the urine of a patient with softening and fragility of the bones. Dr william macintyre was the first person to characterise the precipitate in the urine from a patient with multiple myeloma, and his findings were reported by henry bence jones in a lancet article in 1847. The chemical relations among bencejones proteins, myeloma proteins, and normal. Bencejones proteins are small enough to be filtered out by the kidneys.
Pdf guidelines for the analysis of bence jones protein. These microglobulins, which are synthesized in plasmacell tumors 1, 2, share many immunochemical and physicochemical features with the larger 6. Bence jones proteins are named for henry bence jones, a physician and chemist who first isolated them in 1847. Bence jones protein urine qualitative test for multiple. The hydragel urine profile kit is used for proteinuria investigation by immunofixation on agarose gel in order to. Urine protein electrophoresis upep uses the same principle as spep and has the same advantages and disadvantages. In an attempt to account for antibody specificity and complementarity in terms of structure, human. How the test is performed a cleancatch midstream urine sample is needed. A bence jones protein is a monoclonal globulin protein or immunoglobulin light chain found in the urine, with a molecular weight of 2224 kda. Based on the percentage of igg xchaincontaining molecules in normalserum 35%, the results. Bence jones cast nephropathy myeloma kidney is a type of acute or chronic renal failure resulting from urinary excretion of monoclonal immunoglobulin light chains or bence jones proteins. Immunoelectrophoretic techniques immunofixation allow. Both types of anomalous proteins are often present in cases of multiple myeloma. Bence jones proteins and light chains of immunoglobulins.
Bence jones proteins, also referred to as m proteins, myelomas proteins, paraproteins, and free immunoglobulin light chains, are a component of immunoglobulin produced in excess by b cellderived clonal cell populations. Bence jones proteins are particularly diagnostic of multiple myeloma in the context of target. By definition, the disease results from renal tubular handling of high amounts of bence jones protein. Bencejones proteins, isolated vn of a bencejones protein, and human fii ygglobulin were prepared in albino new zealand rabbits.
Bence jones protein is a monoclonal globulin or immunoglobulin light chain found in the urine, with a molecular weight of 2224 kda. Bencejones protein urine saint lukes health system. Bence jones protein test definition bence jones proteins are small proteins light chains of immunoblobulin found in the urine. A tumor marker is a substance, made by the body, that.
Quantitative bencejones protein test immunoglobulin light chains urine. Purpose bence jones proteins are considered the first tumor marker. Both halves of a bencejones protein were detected in the urine of one patient, and in. Detection of bencejones protein in practice sage journals. Classic punchedout bone lesions in the skull and spine on plain radiographs are pathognomonic for the disease figures 752 and 753. A type of chronic kidney disease often develops, probably as a result of the high concentration of bence jones proteins in the kidney tubules. Although the introduction of electrophoretic methods has led to a much improved characterization of the abnormal globulins which occur in patients with multiple myeloma, the simple determination of the presence of bence jones protein in the urine has remained as important as ever for the diagnosis of multiple myeloma. It is both a practical and a technical improvement. Jeanlouis preudhomme, in encyclopedia of immunology second edition, 1998. Definition a quantitative bencejones protein test measures the specific level of abnormal proteins bencejones proteins in your urine. Laboratory diagnosis of bence jones proteinuria in a patient with. Amino acid sequence studies with bencejones proteins. Bencejones proteins and light chains of inununoglobulins. The detection of bence jones protein in urine by the heat.
Sometimes, when your body makes too many antibodies, the level of light chains also rises. Eight of 35 patients with kappa and 26 with lambda type bj protein had decreased renal function. Alternative names immunoglobulin light chains urine. Or an abnormal bence jones test result may mean you have a type of malignant lymphoma. Pathology multiple myeloma is characterized by excessive numbers of abnormal plasma cells in the bone marrow and overproduction of intact monoclonal immunoglobulin igg, iga, igd, or ige or bence jones protein free monoclonal. Conventionally, proteinuria is diagnosed by a simple dipstick test, although it is possible for the test to give a false negative reading, even with nephrotic range proteinuria if the urine is dilute.
The detection and quantification of monoclonal free light chains in urine bence jones protein, bjp are thorny issues for the laboratorian. The funnel, filter paper, and receiving tube may be heated either in a drying oven or by passing boiling hot water through them several times. Formation of bencejones protein and myeloma protein in. Testing for these proteins is done to diagnose and monitor multiple myeloma and other similar diseases. From our finding of 5 xvi bence jones proteins among 91 xbence jones proteins tested and on the results obtained with the survey of 32 xchaincontaining monoclonal immunoglobulins, we estimate that 5% of xlight chains are of the v,vil subgroup. Bence jones protein test definition of bence jones. Polymeric bence jones proteins in serum in myleoma. Other articles where bencejones protein is discussed. Detection of bence jones protein may be suggestive of multiple myeloma or waldenstroms macroglobulinemia.
Metabolism of bence jones proteins journal of clinical investigation. In order to observe the above in typical form it is necessary to dilute the urine, which is best done by add ing normal urine. Simple method for quantification of bence jones proteins. Bence jones proteins are urinary free light chains detected by urinary protein electrophoresis and immunofixation. Type renal lesions tubular, glomerular or mixed lesion.
If the dilution is made with water the protein will often redissolve badly unless a salt or, better, a few crystals of urea2 are added. Jones proteins concentration according to the literature damaccol waldestrom et. Bence jones protein is a monoclonal free light chain and has much greater signi. Bence jones proteins resulted, in each case, in the formation of a. Bencejones protein definition of bencejones protein by. Understanding and interpreting serum protein electrophoresis. The bencejones protein urine test is used most often to diagnose and check on multiple myeloma, a type of cancer. Bencejones proteins, also referred to as mproteins, myelomas proteins, paraproteins, and free immunoglobulin light chains, are a component of immunoglobulin produced in excess by b cellderived clonal cell populations. Serum protein electrophoresis is used to identify patients with multiple myeloma and other serum protein disorders. Starch gel electrophoresis in 8 m urea of reduced alkylated bencejones proteins yielded patterns of bands corresponding to those of the light l polypeptide chains of the dissociated myeloma protein from the same patient. Some individuals confuse the presence of an intact immunoglobulin monoclonal gammopathy in the urine with a bence jones protein. The bence jones proteins from individual patients were found to correspond in antigenic group to that of the serum myeloma protein. It was not until later that bencejones protein was identi.
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