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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 RBCs. 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]

Classification

Cryoglobulinemia is classically grouped into three types according to the Brouet classification.^[3] Type I is most commonly encountered in patients with a plasma cell dyscrasia such as multiple myeloma or Waldenström macroglobulinemia.^[4] Types II and III are strongly associated with infection by the hepatitis C virus.^[4] 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.^[5]

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.^[6]
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.^[6]
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.^[6]

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.^[6]

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^[7]

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.^[8]^[9] It can also occur in hepatitis B and human immunodeficiency virus infections.^[10] 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 myalgia)^[11] is generally seen with polyclonal CGs seen in essential, viral, or connective tissue disease-associated CG.

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
^ 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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Overview at Mayo Clinic

UpToDate Contents

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1. クリオグロブリンおよびクリオグロブリン血症の概要 overview of cryoglobulins and cryoglobulinemia
2. 混合型クリオグロブリン血症症候群 (本態性混合型クリオグロブリン血症)の臨床症状および診断 clinical manifestations and diagnosis of the mixed cryoglobulinemia syndrome essential mixed cryoglobulinemia
3. 混合型クリオグロブリン血症症候群の治療 treatment of the mixed cryoglobulinemia syndrome
4. Immune-mediated neuropathies
5. C型肝炎ウイルス感染関連腎疾患 renal disease associated with hepatitis c virus infection

English Journal

Cast nephropathy and light-chain deposition disease in waldenström macroglobulinemia.

Gnemmi V, Leleu X, Provot F, Moulonguet F, Buob D.SourceDepartment of Pathology, CHU Lille, Lille, France; Université Lille Nord de France, Lille, France.
American journal of kidney diseases : the official journal of the National Kidney Foundation.Am J Kidney Dis.2012 Sep;60(3):487-91. Epub 2012 Jun 20.
Waldenström macroglobulinemia is a rare low-grade hematologic malignancy due to clonal proliferation of B lymphocytes responsible for immunoglobulin M (IgM) monoclonal gammopathy secreted in serum. This disease is characterized by lymphoplasmacytic tumoral infiltration of bone marrow and various or
PMID 22721930

Low 25-hydroxyvitamin D serum levels correlate with the presence of extra-hepatic manifestations in chronic hepatitis C virus infection.

Terrier B, Jehan F, Munteanu M, Geri G, Saadoun D, Sène D, Poynard T, Souberbielle JC, Cacoub P.SourceDepartment of Internal Medicine, Université Pierre et Marie Curie, Paris 6, Groupe Hospitalier Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), CNRS UMR 7211, Université Pierre et Marie Curie, Paris 6, Groupe Hospitalier Pitié-Salpêtrière, AP-HP, INSERM UMR 561 and Université Paris Diderot, Hôpital Lariboisière, Université Paris 7, Department of Hepatology, Université Pierre et Marie Curie, Paris 6, Groupe Hospitalier Pitié-Salpêtrière, AP-HP and Laboratoire d'explorations fonctionnelles, Université Paris Descartes, Paris 5, Hôpital Necker-Enfants Malades, Paris, France.
Rheumatology (Oxford, England).Rheumatology (Oxford).2012 Aug 20. [Epub ahead of print]
Objective. Chronic HCV infection is associated with extra-hepatic manifestations. Recent studies have suggested an immunomodulatory role for vitamin D during HCV infection. We investigated the association between serum vitamin D status and the presence of HCV extra-hepatic manifestations.Methods. 25
PMID 22908327