Urine Bence Jones Protein Exam

- Bence Jones Proteins are abnormal light chain immunoglobulins of low molecular weight that are derived from the clone of a single plasma cell. This globulin appears in the urine of 50% to 80% of patients with multiple myeloma and in most patients with Waldenstroms macroglobulinemia.

I.

Normal Value and Significance - No Bence Jones proteins are present; presence of Bence Jones proteins in urine suggests multiple myeloma or Waldenstroms macroglobulinemia. High doses of penicillin or aspirin before

collecting the urine can give a false positive result.

II. Purpose - To confirm the presence of multiple myeloma in patients with characteristic signs such as bone pain and persistent anemia and fatigue. III. Indication - Your doctor may order this test when your urine protein level is high

or if you have other signs of multiple myeloma.

- To help diagnose medical conditions that lead to protein in the urine

(proteinuria).
- To diagnose the disease as well as to check how well the disease is

responding to treatment
IV. Procedure A. Pre Procedure

- Explain that the Bence Jones protein test can detect an abnormal protein in the urine - Tell the patient that the test requires an early-morning urine specimen; teach him how to collect a midstream clean catch specimen - If a 24 hour urine specimen is being used, instruct the patient on proper collection of a 24 hour urine specimen B. During Procedure - Confirm the patients identity. - Collect an early morning urine specimen of at least 50 ml - If performing a 24 hour urine collection, collect the patients urine over a 24 hour period, discarding the specimen and retaining the last. C. After Procedure - Keep a 24 hour specimen on ice or in the refrigerator during the collection period - Send the specimen to the laboratory immediately after collection or refrigerate if transport is delayed. If a refrigerated specimen is not analyzed within 24 hours it must be discarded V. Complications - No associated complications

Definition
A quantitative Bence-Jones protein test measures the specific level of abnormal proteins (Bence-Jones proteins) in your urine.

Images:

Male urinary system

Alternative Names
Immunoglobulin light chains - urine; Urine Bence-Jones protein

How the test is performed

Collect a "clean-catch" (midstream) urine sample. To obtain a clean-catch sample, men or boys should wipe clean the head of the penis. Women or girls should wash the area between the labia (lips of the vagina) with soapy water and rinse well. As you start to urinate, allow a small amount to fall into the toilet bowl. This clears the urethra -- the tube that carries urine from the bladder and opens to the outside. Then, in a clean container, catch about 1 to 2 ounces of urine, and remove the container from the urine stream. Give the container to the health care provider or assistant. For an infant: Thoroughly wash the area around the opening of the urethra. Open a urine collection bag (a plastic bag with an adhesive paper on one end), and place it on your infant. For males, the entire penis can be placed in the bag and the adhesive attached to the skin. For females, the bag is placed over the labia. Place a diaper over the infant (bag and all). Check your baby frequently and remove the bag after the infant has urinated into it. For active infants, this procedure may take a couple of attempts -- lively infants can displace the bag, causing an inability to obtain the specimen. The urine is drained into a container for transport back to the health care provider

How the test will feel

The test involves only normal urination, and there is no discomfort.

Why the test is performed

Bence-Jones proteins are relatively small and are filtered out by the kidneys. This test is done to help diagnose medical conditions that lead to protein in the urine (proteinuria). Your doctor may also order this test when urine protein is high and you have other signs that suggest multiple myeloma.

Normal Values
No presence of Bence-Jones proteins is normal.

What abnormal results mean

Bence-Jones proteins are rarely found in urine, but if they are, they are usually associated with multiple myeloma. Less commonly they are present in Waldenstrom's macroglobulinemia, chronic lymphocytic leukemia, oramyloidosis.

Mechanisms of Proteinuria
Normal barriers to protein filtration begin in the glomerulus, which consists of unique capillaries that are permeable to fluid and small solutes but effective barriers to plasma proteins. The adjacent basement membrane and visceral epithelial cells are covered with negatively charged heparan sulfate proteoglycans.5
Most protein-positive dipstick tests Proteins cross to the tubular fluid in inverse proportion to are a result of benign proteinuria, which has no associated morbidity or their size and negative charge. Proteins with a molecular weight of less than 20,000 pass easily across the glomerular mortality.

capillary wall.6 Conversely, albumin, with a molecular weight of 65,000 Daltons and a negative charge, is restricted under normal conditions. The smaller proteins are largely reabsorbed at the proximal tubule, and only small amounts are excreted. The pathophysiologic mechanisms of proteinuria can be classified as glomerular, tubular or overflow (Table 27). Glomerular disease is the most common cause of pathologic proteinuria.8 Several glomerular abnormalities alter the permeability of the glomerular basement membrane, resulting in urinary loss of albumin and immunoglobulins.7 Glomerular malfunction can cause large protein losses; urinary excretion of more than 2 g per 24 hours is usually a result of glomerular disease (Table 3).9

TABLE 2 Classification of Proteinuria

Type Pathophysiologic features Cause Primary or secondary glomerulopathy Tubular or interstitial disease Monoclonal gammopathy, leukemia

TABLE 3 Cause of Proteinuria as Related to Quantity

Daily protein excretion 0.15 to 2.0 g Cause Mild glomerulopathies Tubular proteinuria Overflow proteinuria Usually glomerular Always glomerular

Glomerular Increased glomerular capillary permeability to protein Tubular Decreased tubular reabsorption of proteins in glomerular filtrate Overflow Increased production of lowmolecular-weight proteins

2.0 to 4.0 g >4.0 g

Adapted with permission from McConnell KR, Bia MJ. Evaluation of proteinuria: an approach for the internist. Resident Staff Phys 1994;40:41-8.

Adapted with permission from Abuelo JG. Proteinuria: diagnostic principles and procedures. Ann Intern Med 1983;98:186-91.

Tubular proteinuria occurs when tubulointerstitial disease prevents the proximal tubule from reabsorbing lowmolecular-weight proteins (part of the normal glomerular ultrafiltrate). When a patient has tubular disease, usually less than 2 g of protein is excreted in 24 hours. Tubular diseases include hypertensive nephrosclerosis and tubulointerstitial nephropathy caused by nonsteroidal anti-inflammatory drugs. In overflow proteinuria, low-molecular-weight proteins overwhelm the ability of the proximal tubules to reabsorb filtered proteins. Most often, this is a result of the immunoglobulin overproduction that occurs in multiple myeloma. The resultant light-chain immunoglobulin fragments (Bence Jones proteins) produce a monoclonal spike in the urine electrophoretic pattern. Table 4 lists some common disorders of the three mechanisms of proteinuria.
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A Bence Jones protein is a monoclonal globulin protein found in the blood or urine, with a molecular weight of 22-24 kDa.[1] Finding this protein is often suggestive of multiple myeloma. Bence Jones Proteins are particularly diagnostic of multiple myeloma in the context of end-organ manifestations such as malignant bone marrow cancer, renal failure, lytic bone disease, or 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, often induced by hypercalcemia from the calcium released as the bones are destroyed.[citation needed] The light chains can be detected by heating or electrophoresis of concentrated urine. Light chains precipitate when heated to 50 - 60 degrees C and redisolve at 90 -100 degrees C. These tests are essential in patients suspected of having Bence Jones proteins in their urine as these proteins don't react with the reagents normally utilized in urinalysis dipsticks. This leads to false negative results in people with Bence Jones proteins in their urine undergoing standard urinalysis. There are various rarer conditions that can produce Bence Jones proteins, such as Waldenstrm's macroglobulinemia and other malignances. The Bence Jones protein was described by the English physician Henry Bence Jones in 1847 and published in 1848.[3] The protein was later sequenced by Frank Putnam at the laboratory of Fred Sanger in Cambridge, who was the first to report the entire sequence. However, in case of renal failure or massive synthesis , Bence jones proteins may appear in the blood in significant concentrations .