混合性クリオグロブリン血症
WordNet
- combine (electronic signals); "mixing sounds"
- add as an additional element or part; "mix water into the drink" (同)mix in
- the act of mixing together; "paste made by a mix of flour and water"; "the mixing of sound channels in the recording studio" (同)commixture, admixture, mixture, intermixture, mixing
- an event that combines things in a mixture; "a gradual mixture of cultures" (同)mixture
- a commercially prepared mixture of dry ingredients (同)premix
- to bring or combine together or with something else; "resourcefully he mingled music and dance" (同)mingle, commix, unify, amalgamate
PrepTutorEJDIC
- 〈複数のもの〉‘を'『混ぜ合わせる』,混合する,混同する / …‘を'混ぜ合わせて作る,調合して作る / …‘を'寄せ集める,まとめる / 〈物が〉(…と)混ざる,混合する《+『with』+『名』》 / 〈人が〉(他の人と)交わる,交際する《+『with』+『名』》 / 〈C〉〈U〉混合 / 〈C〉混合物(食品,薬) / 〈C〉〈U〉(水・火を加えて即席にでき上がる)素(もと),ミックス
- (異なった成分(要素)のものが)混ざった,混合の,混成の / 男女混合の / 人種(宗教・国籍など)が違う人々の(から成る)
Wikipedia preview
出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/06/22 15:26:54」(JST)
[Wiki en表示]
| Cryoglobulinemia |
|
| Classification and external resources |
| ICD-10 |
D89.1 |
| ICD-9 |
273.2 |
| DiseasesDB |
3207 |
| MedlinePlus |
000540 |
| eMedicine |
med/480 |
| Patient UK |
Cryoglobulinemia |
| MeSH |
D003449 |
Cryoglobulinemia[1] is a medical condition in which the blood contains large amounts of cryoglobulins – proteins (mostly immunoglobulins themselves) that become insoluble at reduced temperatures. This should be contrasted with cold agglutinins, which cause agglutination of red blood cells. Cryoglobulins typically precipitate at temperatures below normal body temperature (37°C) and will dissolve again if the blood is heated. Cryoglobulinemia can be associated with various diseases such as multiple myeloma and hepatitis C infection.[2] Cryoglobulins usually consist of IgM directed against the Fc region of IgG.[3]
Contents
- 1 Classification
- 2 Causes
- 3 See also
- 4 References
- 5 External links
Classification
Cryoglobulinemia is classically grouped into three types according to the Brouet classification.[4] Type I is most commonly encountered in patients with a plasma cell dyscrasia such as multiple myeloma or Waldenström macroglobulinemia.[5] Types II and III are strongly associated with infection by the hepatitis C virus.[5] Types III are strongly associated with autoimmune disease such as systemic lupus erythematosus and rheumatoid arthritis.
There are three different types of cryoglobulins that have been observed to form in the blood.[6]
| Type |
Composition |
Percent |
Description |
| Type I |
isolated monoclonal immunoglobulins |
10–15% of the total cases |
These are composed of a single monoclonal immunoglobulin paraprotein (usually IgM). Sometimes, these are represented by light chains only and can be extracted from the urine; or, they may accumulate in blood serum in the event of renal failure.[7] |
| Type II |
immunocomplexes formed by monoclonal IgM |
50–60% of reported cases |
They usually have a polyclonal component, usually IgG, and a monoclonal component, usually IgM or IgA, which has an RF function. The IgM can recognize intact IgG or either the Fab region or Fc region of IgG fragments. This is why most type II cryoglobulins are IgM–IgG complexes.[7] |
| Type III |
immunocomplexes formed by polyclonal IgM |
25–30% of the reported cases |
These have very similar function to the type II cryoglobulins; however, they are composed of polyclonal IgM and IgG molecules.[7] |
Types II and III have rheumatoid factor (RF) activity and bind to polyclonal immunoglobulins. These two types are referred to as mixed cryoglobulinemia (MC). When the temperature is raised, the precipitated cryoglobulins will dissolve back into the serum.[7]
In 2006 it was discovered that there are unusual cryoglobulins that show a microheterogeneous composition, with an immunochemical structure that cannot be fit into any of the classifications. A classification of a type II-III variant has been proposed because they are composed of oligoclonal IgMs with traces of polyclonal immunoglobulins[8]
Causes
These proteins may be present in Mycoplasma pneumonia, post streptococcal glomerulonephritis, multiple myeloma, certain leukemias, primary macroglobulinemia, and some autoimmune diseases, such as systemic lupus erythematosus and rheumatoid arthritis. This is also found as a symptom in 35% of chronic hepatitis C infections.[9][10] It can also occur in hepatitis B and human immunodeficiency virus infections.[11] It is important to note that these two different, yet highly representative, clinical syndromes generally reflect different types of underlying CG:
- Hyperviscosity is typically associated with CG due to hematological malignancies and monoclonal immunoglobulins.
- "Meltzer's triad" (purpura, arthralgia and weakness)[12] is generally seen with polyclonal CGs seen in essential, viral, or connective tissue disease-associated CG.
See also
- Cryoglobulinemic purpura
- Hematopoietic ulcer
- Paraproteinemia
References
- ^ "Cryoglobulinemia" at Dorland's Medical Dictionary
- ^ Peveling-Oberhag, J.; Arcaini, L.; Hansmann, M. L.; Zeuzem, S. (2013). "Hepatitis C-associated B-cell non-Hodgkin lymphomas. Epidemiology, molecular signature and clinical management". Journal of Hepatology 59 (1): 169–177. doi:10.1016/j.jhep.2013.03.018. PMID 23542089. edit
- ^ Adam M Tritsch MD. Herbert S Diamond, MD, ed. "Cryoglobulinemia".
- ^ Brouet JC, Clauvel JP, Danon F, Klein M, Seligmann M (1974). "Biologic and clinical significance of cryoglobulins. A report of 86 cases". Am. J. Med. 57 (5): 775–88. doi:10.1016/0002-9343(74)90852-3. PMID 4216269.
- ^ a b Ferri C, Zignego AL, Pileri SA (2002). "Cryoglobulins". J. Clin. Pathol. 55 (1): 4–13. doi:10.1136/jcp.55.1.4. PMC 1769573. PMID 11825916.
- ^ "Cryoglobulin" at Dorland's Medical Dictionary
- ^ a b c d Tedeschi A, Baratè C, Minola E, Morra E (2007). "Cryoglobulinemia". Blood Rev. 21 (4): 183–200. doi:10.1016/j.blre.2006.12.002. PMID 17289231.
- ^ Tissot JD, Schifferli JA, Hochstrasser DF et al. (1994). "Two-dimensional polyacrylamide gel electrophoresis analysis of cryoglobulins and identification of an IgM-associated peptide". J. Immunol. Methods 173 (1): 63–75. doi:10.1016/0022-1759(94)90284-4. PMID 8034987.
- ^ Franco Dammacco, and Domenico Sansonno (September 12, 2013). "Review Article: Therapy for Hepatitis C Virus–Related Cryoglobulinemic Vasculitis". N Engl J Med 369 (11): 1035–1045. doi:10.1056/NEJMra1208642. PMID 24024840.
- ^ Pascual M, Perrin L, Giostra E, Schifferli JA (1990). "Hepatitis C virus in patients with cryoglobulinemia type II". J. Infect. Dis. 162 (2): 569–70. doi:10.1093/infdis/162.2.569. PMID 2115556.
- ^ Liang TJ (May 2009). "Hepatitis B: the virus and disease". Hepatology (Baltimore, Md.) 49 (5 Suppl): S13–21. doi:10.1002/hep.22881. PMC 2809016. PMID 19399811. Retrieved 2011-12-08.
- ^ Monti G, Galli M, Invernizzi F et al. (February 1995). "Cryoglobulinaemias: a multi-centre study of the early clinical and laboratory manifestations of primary and secondary disease. GISC. Italian Group for the Study of Cryoglobulinaemias". QJM 88 (2): 115–26. PMID 7704562.
External links
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Wikimedia Commons has media related to Cryoglobulinemia. |
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Immunoproliferative immunoglobulin disorders (D89, 273)
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| PCDs/PP |
- Plasmacytoma
- Multiple myeloma (Plasma cell leukemia)
- MGUS
- IgM (Macroglobulinemia/Waldenström's macroglobulinemia)
- heavy chain (Heavy chain disease)
- light chain (Primary amyloidosis)
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| Other hypergammaglobulinemia |
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Index of the immune system
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| Description |
- Physiology
- cells
- autoantigens
- autoantibodies
- complement
- surface antigens
- IG receptors
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| Disease |
- Allergies
- Immunodeficiency
- Immunoproliferative immunoglobulin disorders
- Hypersensitivity and autoimmune disorders
- Neoplasms and cancer
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| Treatment |
- Procedures
- Drugs
- antihistamines
- immunostimulants
- immunosuppressants
- monoclonal antibodies
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UpToDate Contents
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English Journal
- Hepatitis C virus as a systemic disease: reaching beyond the liver.
- Gill K1, Ghazinian H2,3, Manch R1, Gish R4,5.
- Hepatology international.Hepatol Int.2015 Dec 10. [Epub ahead of print]
- Chronic hepatitis C (CHC) is associated with multiple extrahepatic manifestations that may impact infected patients. The mechanisms through which these develop include those which are immunological, in which the chronic persistence of virus leads to the circulation of immune complexes (mixed cryoglo
- PMID 26660706
- Extrahepatic Manifestations of Hepatitis C Virus.
- Viganò M1, Colombo M2.
- Gastroenterology clinics of North America.Gastroenterol Clin North Am.2015 Dec;44(4):775-91. doi: 10.1016/j.gtc.2015.07.006. Epub 2015 Aug 12.
- Chronic infection with the hepatitis C virus (HCV) is a major cause of liver disease worldwide and is also responsible for extrahepatic manifestations (EHMs) involving the skin, kidneys, salivary glands, eyes, thyroid, and immune system. Mixed cryoglobulinemia is the prototype EHM related to HCV inf
- PMID 26600219
- Non HCV-related infectious cryoglobulinemia vasculitis: Results from the French nationwide CryoVas survey and systematic review of the literature.
- Terrier B1, Marie I2, Lacraz A3, Belenotti P4, Bonnet F5, Chiche L6, Graffin B7, Hot A8, Kahn JE9, Michel C10, Quemeneur T11, de Saint-Martin L12, Hermine O13, Léger JM14, Mariette X15, Senet P16, Plaisier E17, Cacoub P18.
- Journal of autoimmunity.J Autoimmun.2015 Dec;65:74-81. doi: 10.1016/j.jaut.2015.08.008. Epub 2015 Aug 29.
- In patients with infectious cryoglobulinemia vasculitis (CryoVas) in the absence of hepatitis C virus infection, data on presentation, therapeutic management and outcome are lacking. We conducted a nationwide survey that included patients with HCV-negative CryoVas. We describe here the presentation,
- PMID 26320984
Japanese Journal
- Pulmonary Extranodal Marginal Zone Lymphoma with Macroglobulinemia and Mixed Cryoglobulinemia Developed in a Patient with Chronic Hepatitis C
- Min Chisun,Higuchi Takakazu,Koyamada Ryosuke,Yamaguchi Norihiro,Okada Sadamu
- Internal Medicine 54(16), 2061-2064, 2015
- … We report a 65-year-old woman with a chronic hepatitis C virus infection who developed pulmonary extranodal marginal zone lymphoma (EMZL) of mucosa-associated lymphoid tissues complicated with macroglobulinemia and mixed cryoglobulinemia. …
- NAID 130005092921
- The J6JFH1 Strain of Hepatitis C Virus Infects Human B-Cells with Low Replication Efficacy
- Nakai Masato,Seya Tsukasa,Matsumoto Misako,Shimotohno Kunitada,Sakamoto Naoya,Aly Hussein H.
- Viral Immunology 27(6), 285-294, 2014-08
- … However, extrahepatic manifestations such as mixed cryoglobulinemia, a systemic immune complex-mediated disorder characterized by B-cell proliferation, which may evolve into overt B-cell non-Hodgkin's lymphoma, have been demonstrated. …
- NAID 120005468145
- High Level of Rheumatoid Factor is Associated with Hepatitis B Viremia in Patients with Chronic Hepatitis B
- Arai Jun,Ito Takayoshi,Miyashita Miyuki,Sshimoduma Yuu,Uchikoshi Manabu,Morikawa Kenichi,Eguchi Junichi,Hayashi Eiichi,Nozawa Hisako,Yoshida Hitoshi
- Showa Univ J Med Sci 26(1), 75-83, 2014-03
- … However these viruses are also associated with lymphoproliferative disorders (LPDs), such as essential mixed cryoglobulinemia and B-cell non-Hodgkin's lymphoma. … LPD markers, such as cryoglobulinemia, high levels of rheumatoid factor (RF), hypocomplementemia, and B cell clonality, were measured and analyzed along with viral factors. …
- NAID 120005536501
Related Links
- Type I cryoglobulinemia, or simple cryoglobulinemia, is the result of a monoclonal immunoglobulin, usually immunoglobulin M (IgM) or, less frequently ... Types II and III cryoglobulinemia (mixed cryoglobulinemia) contain ...
- Read about essential mixed cryoglobulinemia causes, symptoms (joint pain, swelling, enlarged spleen, skin vasculitis, nerve & kidney disease), prognosis and treatment. ... Kidney Failure Kidney failure can occur from an acute event ...
Related Pictures
★リンクテーブル★
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- 英
- mixed cryoglobulinemia
- 関
- クリオグロブリン血症
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- admix、admixture、blend、intermingle、merge、mixture、scramble、shuffle、shuffling
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- combined
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クリオグロブリン血症