WordNet

any of a large group of nitrogenous organic compounds that are essential constituents of living cells; consist of polymers of amino acids; essential in the diet of animals for growth and for repair of tissues; can be obtained from meat and eggs and milk and legumes; "a diet high in protein"
a tumor of the bone marrow (usually malignant) composed of cells normally found in bone marrow

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/10/27 13:24:19」(JST)

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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. 多発性骨髄腫の病理生物学 pathobiology of multiple myeloma
2. 多発性骨髄腫患者のマネージメントの概要 overview of the management of multiple myeloma
3. 多発性骨髄腫における病期分類および予後研究 staging and prognostic studies in multiple myeloma
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. 多発性骨髄腫の治療に対する奏効性評価 evaluating response to treatment of multiple myeloma

English Journal

Low immunoglobulin A levels detected via the tissue transglutaminase assay can reveal previously undetected monoclonal proteins.

Wallage M1, Dutton D2, Lock RJ3.
Annals of clinical biochemistry.Ann Clin Biochem.2016 May;53(Pt 3):409-11. doi: 10.1177/0004563215625692. Epub 2015 Dec 17.
Increased awareness of coeliac disease and the 2009 NICE guidance has led to an increase in patients being screened for Immunoglobulin A deficiency. We have shown previously that this provides an opportunity for the early identification of other underlying primary immunodeficiency, e.g. common varia
PMID 26684021

Exosomes promote bone marrow angiogenesis in hematologic neoplasia: the role of hypoxia.

Ohyashiki JH1, Umezu T, Ohyashiki K.
Current opinion in hematology.Curr Opin Hematol.2016 May;23(3):268-73. doi: 10.1097/MOH.0000000000000235.
PURPOSE OF REVIEW: To review the data on angiogenesis related to exosomes secreted by tumor cells in hematologic neoplasia and to elucidate the role of exosomes and exosomal miRNA in the bone marrow microenvironment, especially under hypoxic conditions.RECENT FINDINGS: Cross-talk between bone marrow
PMID 26866730

Clinicopathological characteristics and outcomes of light chain deposition disease: an analysis of 48 patients in a single Chinese center.

Li XM1, Rui HC1, Liang DD1, Xu F1, Liang SS1, Zhu XD1, Huang XH1, Liu ZH1, Zeng CH2.
Annals of hematology.Ann Hematol.2016 Apr 8. [Epub ahead of print]
To explore the clinicopathological characteristics and outcomes of light chain deposition disease (LCDD) in a Chinese population, we retrospectively studied the clinicopathological data, treatment, and outcomes of 48 patients with biopsy-proven LCDD from a single center. Among the patients, there we
PMID 27056200

Japanese Journal

Anaplastic Atypical Myeloma with Extensive Cutaneous Involvement in a Dog

FUKUMOTO Shinya,HANAZONO Kiwamu,KAWASAKI Naomi,HORI Yasutomo,HIGUCHI Seiichi,SASAKI Takushi,TEMMA Kyosuke,UCHIDE Tsuyoshi
Journal of Veterinary Medical Science advpub(0), 1108240613, 2011
… Observation of bone marrow plasmacytosis, osteolytic bone lesions, serum myeloma protein and cutaneous infiltration of myeloma cells led us to a diagnosis of multiple myeloma (MM) with cutaneous involvement. …
NAID 130001032918

肝機能異常を伴った多発性骨髄腫の1例

斎藤広信,高橋敦史,阿部和道,物江恭子,菅野有紀子,横川順子,大平弘正
肝臓 52(1), 65-69, 2011
症例は65歳，男性．両肩関節痛を認め，近医整形外科で受診し，変形性肩関節症と診断された．1カ月後から両膝・両手首の関節痛も出現し，肝機能異常と貧血も認めたため入院した．免疫グロブリンの低下およびカルシウム値の増加から血液疾患を疑い，骨髄穿刺検査を施行した．骨髄検査ではCD138陽性の異型形質細胞がびまん性に増生し，造血細胞巣の70％以上を占め，頭部レントゲン検査での打ち抜き像，尿中Bence Jo …
NAID 130000445327