パラプロテイン血症
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Paraproteinemia |
Classification and external resources |
ICD-10 |
D89.2 |
ICD-9-CM |
273.1 - 273.2 |
DiseasesDB |
9614 |
Patient UK |
Paraproteinemia |
MeSH |
D010265 |
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]
Contents
- 1 Types
- 2 Possible causes
- 3 Diagnosis
- 4 See also
- 5 References
- 6 External links
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]
See also
- Monoclonal gammopathy of undetermined significance
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.
Immunoproliferative immunoglobulin disorders (D89, 273)
|
|
PCDs/PP |
- Plasmacytoma
- Multiple myeloma (Plasma cell leukemia)
- MGUS
- IgM (Macroglobulinemia/Waldenström's macroglobulinemia)
- heavy chain (Heavy chain disease)
- light chain (Primary amyloidosis)
|
|
Other hypergammaglobulinemia |
|
|
Index of the immune system
|
|
Description |
- Physiology
- cells
- autoantigens
- autoantibodies
- complement
- surface antigens
- IG receptors
|
|
Disease |
- Allergies
- Immunodeficiency
- Immunoproliferative immunoglobulin disorders
- Hypersensitivity and autoimmune disorders
- Neoplasms and cancer
|
|
Treatment |
- Procedures
- Drugs
- antihistamines
- immunostimulants
- immunosuppressants
- monoclonal antibodies
|
|
|
Hematological malignancy/leukemia histology (ICD-O 9590–9989, C81–C96, 200–208)
Lymphoid/Lymphoproliferative, Lymphomas/Lymphoid leukemias (9590–9739, 9800–9839)
|
|
B cell
(lymphoma,
leukemia)
(most CD19
|
By development/
marker |
TdT+ |
- ALL (Precursor B acute lymphoblastic leukemia/lymphoma)
|
|
CD5+ |
- mantle zone (Mantle cell)
|
|
CD22+ |
- Prolymphocytic
- CD11c+ (Hairy cell leukemia)
|
|
CD79a+ |
- germinal center/follicular B cell (Follicular
- Burkitt's
- GCB DLBCL
- Primary cutaneous follicular lymphoma)
- marginal zone/marginal-zone B cell (Splenic marginal zone
- MALT
- Nodal marginal zone
- Primary cutaneous marginal zone lymphoma)
|
|
RS (CD15+, CD30+) |
- Classic Hodgkin's lymphoma (Nodular sclerosis)
- CD20+ (Nodular lymphocyte predominant Hodgkin's lymphoma)
|
|
PCDs/PP
(CD38+/CD138+) |
- see immunoproliferative immunoglobulin disorders
|
|
|
By infection |
- KSHV (Primary effusion)
- EBV (Lymphomatoid granulomatosis
- Post-transplant lymphoproliferative disorder)
- HIV (AIDS-related lymphoma)
- Helicobacter pylori (MALT lymphoma)
|
|
Cutaneous |
- Diffuse large B-cell lymphoma
- Intravascular large B-cell lymphoma
- Primary cutaneous marginal zone lymphoma
- Primary cutaneous immunocytoma
- Plasmacytoma
- Plasmacytosis
- Primary cutaneous follicular lymphoma
|
|
|
T/NK |
T cell
(lymphoma,
leukemia)
(most CD3
|
By development/
marker |
- TdT+: ALL (Precursor T acute lymphoblastic leukemia/lymphoma)
- prolymphocyte (Prolymphocytic)
- CD30+ (Anaplastic large-cell lymphoma
- Lymphomatoid papulosis type A)
|
|
Cutaneous |
MF+variants |
- indolent: Mycosis fungoides
- Pagetoid reticulosis
- Granulomatous slack skin
aggressive: Sézary disease
- Adult T-cell leukemia/lymphoma
|
|
Non-MF |
- CD30-: Non-mycosis fungoides CD30− cutaneous large T-cell lymphoma
- Pleomorphic T-cell lymphoma
- Lymphomatoid papulosis type B
- CD30+: CD30+ cutaneous T-cell lymphoma
- Secondary cutaneous CD30+ large cell lymphoma
- Lymphomatoid papulosis type A
|
|
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Other peripheral |
- Hepatosplenic
- Angioimmunoblastic
- Enteropathy-associated T-cell lymphoma
- Peripheral T-cell lymphoma-Not-Otherwise-Specified (Lennert lymphoma)
- Subcutaneous T-cell lymphoma
|
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By infection |
- HTLV-1 (Adult T-cell leukemia/lymphoma)
|
|
|
NK cell/
(most CD56) |
- Aggressive NK-cell leukemia
- Blastic NK cell lymphoma
|
|
T or NK |
- EBV (Extranodal NK-T-cell lymphoma/Angiocentric lymphoma)
- Large granular lymphocytic leukemia
|
|
|
Lymphoid+myeloid |
- Acute biphenotypic leukaemia
|
|
Lymphocytosis |
- Lymphoproliferative disorders (X-linked lymphoproliferative disease
- Autoimmune lymphoproliferative syndrome)
- Leukemoid reaction
- Diffuse infiltrative lymphocytosis syndrome
|
|
|
Cutaneous lymphoid hyperplasia |
- Cutaneous lymphoid hyperplasia
- with bandlike and perivascular patterns
- with nodular pattern
- Jessner lymphocytic infiltrate of the skin
|
|
Index of the immune system
|
|
Description |
- Physiology
- cells
- autoantigens
- autoantibodies
- complement
- surface antigens
- IG receptors
|
|
Disease |
- Allergies
- Immunodeficiency
- Immunoproliferative immunoglobulin disorders
- Hypersensitivity and autoimmune disorders
- Neoplasms and cancer
|
|
Treatment |
- Procedures
- Drugs
- antihistamines
- immunostimulants
- immunosuppressants
- monoclonal antibodies
|
|
|
UpToDate Contents
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English Journal
- Scleroderma renal crisis-like acute renal failure associated with mucopolysaccharide accumulation in renal vessels in a patient with scleromyxedema.
- Lee YH, Sahu J, O'Brien MS, D'Agati VD, Jimenez SA.SourceFrom the *Jefferson Institute of Molecular Medicine and Division of Connective Tissue Diseases, Department of Dermatology and Cutaneous Biology, Thomas Jefferson University, Philadelphia, PA; †Department of Arthritis and Rheumatology, Lehigh Valley Physician Group, Allentown, PA; and ‡Department of Pathology, College of Physicians and Surgeons, Columbia University, New York, NY.
- Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases.J Clin Rheumatol.2011 Sep;17(6):318-22.
- Scleromyxedema is a systemic disease characterized by lichenoid papules, nodules, and plaques on the skin and often diffuse skin induration resembling the cutaneous involvement of systemic sclerosis. The systemic involvement affects the musculoskeletal, pulmonary, cardiovascular, gastrointestinal, a
- PMID 21869708
- Localized Primary Cutaneous Nodular Amyloidosis in a Patient With Paraproteinemia.
- Villar M, Burgues M, Rodriguez-Peralto JL, Rivera R, Vanaclocha F.SourceServicio de Dermatologia, Hospital Universitario 12 de octubre, Madrid, Espana.
- Actas dermo-sifiliograficas.Actas Dermosifiliogr.2011 Aug 30. [Epub ahead of print]
- PMID 21885025
Japanese Journal
- The role of sulfoglucuronosyl glycosphingolipids in the pathogenesis of monoclonal IgM paraproteinemia and peripheral neuropathy
- Ariga Toshio
- Proceedings of the Japan Academy. Ser. B, Physical and Biological Sciences 87(7), 386-404, 2011-07
- NAID 40018937044
- パラプロテイン血症 (特集 腎障害をきたす全身性疾患--最近の進歩)
Related Links
- Paraproteinemia, or monoclonal gammopathy, is the presence of excessive amounts of a single monoclonal gammaglobulin (in this case denominated " paraprotein") in the blood. It denotes an underlying immunoproliferative disorder. ...
Related Pictures
★リンクテーブル★
[★]
- 英
- paraproteinemia
- 同
- パラプロテイネミア
- 関
- 異常蛋白血症。単クローン性免疫グロブリン血症と同じ意味?
[★]
- 英
- paraproteinemia
- 関
- 単クローン性γグロブリン血症、パラプロテイン血症、形質細胞疾患
[★]
IgAパラプロテイン血症