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Paraproteinemia, or monoclonal gammopathy, is the presence of excessive amounts of paraprotein or single monoclonal gammaglobulin in the blood. It is usually due to an underlying immunoproliferative disorder.

It is sometimes considered equivalent to plasma cell dyscrasia.^[1]

Types

Paraproteinemias may be categorized according to the type of monoclonal protein found in blood:

Light chains only (or Bence Jones protein). This may be associated with multiple myeloma or AL amyloidosis.
Heavy chains only (also known as "heavy chain disease");
Whole immunoglobulins. In this case, the paraprotein goes under the name of "M-protein" ("M" for monoclonal). If immunoglobulins tend to precipitate within blood vessels with cold, that phenomenon takes the name of cryoglobulinaemia.

The three types of paraproteins may occur alone or in combination in a given individual. Note that while most heavy chains or whole immunoglobulins remain within blood vessels, light chains frequently escape and are excreted by the kidneys into urine, where they take the name of Bence Jones protein.

It is also possible for paraproteins (usually whole immunoglobulins) to form polymers by aggregating with each other; this takes the name of macroglobulinemia and may lead to further complications. For example, certain macroglobulins tend to precipitate within blood vessel with cold, a phenomenon known as cryoglobulinemia. Others may make blood too viscous to flow smoothly (usually with IgM macroglobulins), a phenomenon known as Waldenström macroglobulinemia.

Possible causes

Leukemias and lymphomas of various types, but usually B-cell Non-Hodgkin lymphomas with a plasma cell component.
- Myeloma
- Plasmacytoma
- Lymphoplasmacytic lymphoma

Idiopathic (no discernible cause): some of these will be revealed as leukemias or lymphomas over the years.
- Monoclonal gammopathy of undetermined significance
- Primary AL amyloidosis (light chains only)

Diagnosis

These disorders are characterized by the presence of any abnormal protein that is involved in the immune system, which are most often immunoglobulins and are associated with the clonal proliferation of lymphocytes.^[2]

When a paraproteinemia is present in the blood, there will be a narrow band, or spike, in the serum protein electrophoresis because there will be an excess of production of one protein.^[3]

There are two large classes of blood proteins: albumin and globulin. They are generally equal in proportion, but albumin is much smaller than globulin, and slightly negatively charged, which leads to an accumulation at the end of the electrophoretic gel. The globulins separate out into three regions on the electrophoretic gel, which are the α band, the β band, and the γ band.

The α band can be separated into two components: α1 and α2. The α1 region consists mostly of α1-antitrypsin and α1-acid glycoprotein. The α2 region is mostly haptoglobin, α2-macroglobulin, α2-antiplasmin and ceruloplasmin.

The β band consists of transferrin, low-density lipoproteins, and complement system proteins.^[4]

The γ band is where the immunoglobulins appear, which is why they are also known as gammaglobulins.^[5] The majority of paraproteins appear in this band.^[4]

References

^ "paraproteinemia" at Dorland's Medical Dictionary
^ Health Communication Network. Immunoproliferative disorders- Topic Tree. http://www.use.hcn.com.au/subject.%60Immunoproliferative%20Disorders%60/home.html. Accessed March 2007.
^ Ma ES, Lee ET (2007). "A case of IgM paraproteinemia in which serum free light chain values were within reference intervals". Clin. Chem. 53 (2): 362–3. doi:10.1373/clinchem.2006.080317. PMID 17259251.
^ ^a ^b Martínez-Gómez MA, Carril-Avilés MM, Sagrado S, Villanueva-Camañas RM, Medina-Hernández MJ (2007). "Characterization of antihistamine-human serum protein interactions by capillary electrophoresis". J Chromatogr A 1147 (2): 261–9. doi:10.1016/j.chroma.2007.02.054. PMID 17339039.
^ Abbas, A.K and Lichtman, A.H. Cellular and Molecular Immunology. Fifth Edition. Elsevier Saunders. Philadelphia. 2005

External links

Paraproteinaemia at patient.co.uk.

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. モノクローナル蛋白の認識 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マネージメント 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. 壊疽性膿皮症：病因、臨床症状、および診断 pyoderma gangrenosum pathogenesis clinical features and diagnosis

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