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Serum protein electrophoresis showing a paraprotein (peak in the gamma zone) in a patient with multiple myeloma.

A myeloma protein is an abnormal immunoglobulin fragment or immunoglobulin light chain that is produced in excess by an abnormal clonal proliferation of plasma cells, typically in multiple myeloma. Other terms for such a protein are M protein, M component, spike protein, or paraprotein. This proliferation of the myeloma protein has several deleterious effects on the body, including impaired immune function, abnormally high viscosity ("thickness") of the blood, and kidney damage.

Myeloma is a malignancy of the plasma cell. Plasma cells produce immunoglobulins, which are commonly called antibodies. There are literally thousands of different antibodies, each consisting of pairs of heavy and light chains. Antibodies are typically grouped into five types: IgA, IgD, IgE, IgG, and IgM. When someone contracts myeloma, a malignant clone, a rogue plasma cell, reproduces in an uncontrolled fashion, resulting in overproduction of the specific antibody that it was designed to produce. Each type of antibody has a different number of light chain and heavy chain pairs. As a result, there is a characteristic normal distribution of these antibodies in the blood by molecular weight. When there is a malignant clone, there is usually overproduction of a single antibody, resulting in a "spike" on the normal distribution, which is called an M spike (or monoclonal spike). People will sometimes develop a condition called MGUS (Monoclonal gammopathy of undetermined significance), where there is overproduction of one antibody but the condition is benign (does not threaten the patient's health). An explanation of the difference between multiple myeloma and MGUS can be found in the International Myeloma Foundation's Patient Handbook^[1] and Concise Review^[2]

Detection of paraproteins in the urine or blood is most often associated with benign monoclonal gammopathy of undetermined significance (MGUS), where they remain "silent",^[3] and multiple myeloma. An excess in the blood is known as paraproteinemia. Paraproteins form a narrow band, or 'spike' in protein electrophoresis as they are all exactly the same protein. Unlike normal immunoglobulin antibodies, paraproteins cannot fight infection.

Serum free light-chain measurement can detect free light chains in the blood. Monoclonal free light chains in the serum or urine are called Bence Jones proteins.

History

The concept and the term paraprotein were introduced by the Berlin pathologist Dr Kurt Apitz in 1940,^[4] at that time the Oberarzt of the pathological institute at the Charité hospital.^[5]

Paraproteins allowed the detailed study of immunoglobulins, which eventually led to the production of monoclonal antibodies in 1975.

Interpretation upon detection

Blood serum paraprotein levels of more than 30 g/L is diagnostic of multiple myeloma, according to the diagnostic criteria of the International Myeloma Working Group,^[6] which were updated in 2009.^[7] Detection of paraprotein in serum of less than 30 g/L is classified as monoclonal gammopathy of undetermined significance in cases where clonal plasma cells constitute less than 10% on bone marrow biopsy and there are no myeloma-related organ or tissue impairment.^[6]^[7]

References

^ "Multiple Myeloma Patient Handbook". International Myeloma Foundation.
^ "Multiple Myeloma Concise Review". International Myeloma Foundation.
^ Maniatis A (1998). "Pathophysiology of paraprotein production.". Ren Fail 20 (6): 821–8. doi:10.3109/08860229809045179. PMID 9834980.
^ Apitz K. Die Paraproteinosen. Über die Störungen des Eiweißstoffwechsels bei Plasmozytomen. Virchows Arch Pathol Anat 1940;306:630-699.
^ McDevitt HO. Albert Hewett Coons. In: "Biographical Memoirs", National Academy of Sciences 1996;69:26-37. ISBN 0-309-05346-3. Fulltext.
^ ^a ^b International Myeloma Working Group (2003). "Criteria for the classification of monoclonal gammopathies, multiple myeloma and related disorders: a report of the International Myeloma Working Group". Br. J. Haematol. 121 (5): 749–57. doi:10.1046/j.1365-2141.2003.04355.x. PMID 12780789.
^ ^a ^b Kyle RA, Rajkumar SV (January 2009). "Criteria for diagnosis, staging, risk stratification and response assessment of multiple myeloma". Leukemia 23 (1): 3–9. doi:10.1038/leu.2008.291. PMC 2627786. PMID 18971951.

External links

Paraproteins at the US National Library of Medicine Medical Subject Headings (MeSH)
Educational Resource for Paraproteins
Paraprotein typing

UpToDate Contents

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1. 意義未確定の単クローン性免疫グロブリン血症（MGUS）の診断 diagnosis of monoclonal gammopathy of undetermined significance
2. モノクローナルタンパクの認識 recognition of monoclonal proteins
3. 意義不明の単クローン性免疫グロブリン血症の臨床経過および管理 clinical course and management of monoclonal gammopathy of undetermined significance
4. 多発性骨髄腫の臨床的特徴、検査所見、および診断 clinical features laboratory manifestations and diagnosis of multiple myeloma
5. ワルデンシュトレーム型マクログロブリン血症の疫学、病因、臨床症状、および診断 epidemiology pathogenesis clinical manifestations and diagnosis of waldenstrom macroglobulinemia

English Journal

Techniques and applications of perioperative therapeutic plasma exchange.

Roman PE, Devore AD, Welsby IJ.Author information aDepartment of Anesthesiology, University of Maryland, Baltimore, Maryland bDepartment of Anesthesiology, Duke University Medical Center, Durham cDivision of Cardiology, Duke University Medical Center, Durham, North Carolina, USA.AbstractPURPOSE OF REVIEW: Therapeutic plasma exchange (TPE) is a useful adjunct in the management of antibody-mediated disorders. The indications for TPE now include the perioperative setting. This review updates the anesthesiologist on the relevant clinical indications and precautions of plasma exchange.
Current opinion in anaesthesiology.Curr Opin Anaesthesiol.2014 Feb;27(1):57-64. doi: 10.1097/ACO.0000000000000037.
PURPOSE OF REVIEW: Therapeutic plasma exchange (TPE) is a useful adjunct in the management of antibody-mediated disorders. The indications for TPE now include the perioperative setting. This review updates the anesthesiologist on the relevant clinical indications and precautions of plasma exchange.R
PMID 24335592

The Effect of Paraprotein on Platelet Aggregation.

Djunic I, Elezovic I, Ilic V, Milosevic-Jovcic N, Bila J, Suvajdzic-Vukovic N, Antic D, Vidovic A, Tomin D.Author information Clinic for Hematology, Clinical Center of Serbia, Belgrade, Serbia.AbstractBACKGROUND: Some patients with paraproteinemia have platelet aggregation disorders and the aim of this study was to examine disturbance of platelet aggregation in healthy blood donors by isolated paraprotein in vitro.
Journal of clinical laboratory analysis.J Clin Lab Anal.2014 Jan 6. doi: 10.1002/jcla.21658. [Epub ahead of print]
BACKGROUND: Some patients with paraproteinemia have platelet aggregation disorders and the aim of this study was to examine disturbance of platelet aggregation in healthy blood donors by isolated paraprotein in vitro.METHODS: Using Rivanol, paraprotein was separated from the serum of ten patients wi
PMID 24395751

Principles of separation: indications and therapeutic targets for plasma exchange.

Williams ME, Balogun RA.Author information Renal Division, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts, †Division of Nephrology, University of Virginia, Charlottesville, Virginia.AbstractExtracorporeal "blood purification," mainly in the form of hemodialysis has been a major portion of the clinical activity of many nephrologists for the past 5 decades. A possibly older procedure, therapeutic plasma exchange, separates and then removes plasma as a method of removing pathogenic material from the patient. In contrast to hemodialysis, therapeutic plasma exchange preferentially removes biologic substances of high molecular weight such as autoantibodies or alloantibodies, antigen-antibody complexes, and Ig paraproteins. These molecular targets may be cleared through two alternative procedures: centrifugal separation and membrane separation. This review presents operational features of each procedure, with relevance to the nephrologist. Kinetics of removal of these plasma constituents are based on the principles of separation by the apheresis technique and by features specific to each molecular target, including their production and compartmentalization in the body. Molecular targets for common renal conditions requiring therapeutic plasma exchange are also discussed in detail.
Clinical journal of the American Society of Nephrology : CJASN.Clin J Am Soc Nephrol.2014 Jan;9(1):181-90. doi: 10.2215/CJN.04680513. Epub 2013 Oct 31.
Extracorporeal "blood purification," mainly in the form of hemodialysis has been a major portion of the clinical activity of many nephrologists for the past 5 decades. A possibly older procedure, therapeutic plasma exchange, separates and then removes plasma as a method of removing pathogenic materi
PMID 24178973