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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 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]

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 myalgia)^[12] 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
^ Template:Cite url=http://emedicine.medscape.com/article/329255-overview
^ 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

Wikimedia Commons has media related to Cryoglobulinemia.

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. Overview of renal disease associated with hepatitis C virus infection
5. クリオフィブリノゲン血症 cryofibrinogenemia

English Journal

α2-HS glycoprotein is an essential component of cryoglobulin associated with chronic hepatitis C.

Okazaki T, Yamazaki K, Iwasaki T, Okano T, Kurosaki Y, Nakamura K, Fujioka T, Yotsuyanagi H.
Clinical chemistry and laboratory medicine : CCLM / FESCC.Clin Chem Lab Med.2013 Feb 1;51(2):e17-8. doi: 10.1515/cclm-2012-0393.
PMID 23348219

Cold-Induced Precipitation of a Monoclonal IgM: A Negative Activation Enthalpy Reaction.

Meliga SC, Farrugia W, Ramsland PA, Falconer RJ.SourceAustralian Institute for Bioengineering and Nanotechnology, University of Queensland , St. Lucia, Qld 4072, Australia.
The journal of physical chemistry. B.J Phys Chem B.2013 Jan 17;117(2):490-4. doi: 10.1021/jp309109k. Epub 2013 Jan 4.
Cold-induced precipitation of a monoclonal IgM cryoglobulin isolated from a patient with Waldenström's macroglobulinemia was observed to have a negative activation enthalpy. The rate of the reaction increased, as the temperature decreased. Differential scanning calorimetry of the monoclonal IgM sho
PMID 23240622

Japanese Journal

症例報告クリオグロブリン血症に対してレナリドミド・デキサメサゾン併用療法が有効であった多発性骨髄腫

谷口広明,今泉芳孝,牧山純也 [他]
臨床血液 55(8), 953-957, 2014-08
NAID 40020195589

クリオグロブリン血症に対してレナリドミド・デキサメサゾン併用療法が有効であった多発性骨髄腫

谷口広明,今泉芳孝,牧山純也,安東恒史,澤山靖,今西大介,田口潤,対馬秀樹,波多智子,宮﨑泰司
臨床血液 55(8), 953-957, 2014
クリオグロブリン血症(Cg)を合併した多発性骨髄腫(MM)に対する標準治療は確立していない。今回レナリドミドとデキサメサゾンの併用療法（Ld療法）が有効であった症例を経験した。症例は76歳，女性。主訴は右踵部の皮膚潰瘍，四肢の疼痛としびれであった。20年前にCg併発MMと診断された。以降MMに対しては従来の抗癌剤治療，そしてCg関連症状に対しては血漿交換療法など行われていたが，Cg関連症状のコント …
NAID 130004688009

Hepatitis C Virus-associated Neuropathy Accompanied by Eosinophilic Vasculitis and Granuloma Formation

Yuki Natsuko,Yoshioka Akira,Yasuda Rei,Ohmichi Takuma,Oka Nobuyuki
Internal Medicine 53(11), 1187-1190, 2014
… The plasma HCV viral load was high, and cryoglobulin was positive. …
NAID 130004466339

Related Pictures

★リンクテーブル★

リンク元	「クリオグロブリン」「Cry」
拡張検索	「cryoglobulinemic vasculitis」「cryoglobulinemia」

「クリオグロブリン」

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

　　[★]

英: cryoglobulin, Cry
関: 低温型グロブリン

概念

血清を低温(4℃)に保存すると、白色またはゲル状化し、37℃に戻すと再融解するという可逆的変化を示す。

分類

タイプI：Mタンパク
タイプII：Mタンパク+多クローン性免疫グロブリン
タイプIII：多クローン性免疫グロブリン

LAB.755

	タイプI	タイプII	タイプIII
多発性骨髄腫	・
原発性マクログロブリン血症	・	・
慢性リンパ性白血病		・	・
全身性エリテマトーデス			・
結節性動脈周囲炎			・
シェーグレン症候群		・	・
関節リウマチ		・	・
リウマチ熱		・	○
感染性心内膜炎		・	○
C型肝炎		○	・
本態性クリオグロブリン血症	・	・	○

検査に与える影響

37℃以下で沈殿するとき補体を取り込むため補体価が低下する。

「Cry」

　　[★]

「cryoglobulinemic vasculitis」

　　[★] クリオグロブリン血症性血管炎

「cryoglobulinemia」

　　[★] クリオグロブリン血症

[1] "Cryoglobulinemia" at Dorland's Medical Dictionary

[Peveling-Oberhag-2] 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

[3] Template:Cite url=http://emedicine.medscape.com/article/329255-overview

[pmid4216269-4] 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.

[Ferri2002-5] 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.

[6] "Cryoglobulin" at Dorland's Medical Dictionary

[item11-7] 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.

[item12-8] 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.

[NEJM2013-9] 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.

[10] 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.

[pmid19399811-11] 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.

[pmid7704562-12] 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.

匿名

検索

案内

案内

cryoglobulin