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Cryoglobulinemia
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Cryoglobulinemia
Classification and external resources

ICD-10

D89.1

ICD-9

273.2

DiseasesDB

3207

MedlinePlus

000540

eMedicine

med/480

MeSH

D003449

Cryoglobulinemia[1] is a medical condition in which the blood contains large amounts of cryoglobulins - proteins that become insoluble at reduced temperatures. Cryoglobulins typically precipitate at temperatures below normal body temperature (37 degrees Celsius) and will dissolve again if the blood is heated. Cryoglobulinemia can be associated with various diseases such as multiple myeloma and hepatitis C infection.

Contents
[hide]

1 Classification 2 Causes 3 See also 4 References 5 External links

[edit] Classification
Cryoglobulinemia is classically grouped into three types according to the Brouet classification.[2] Type I is most commonly encountered in patients with a plasma cell dyscrasia such as multiple myeloma or Waldenstrm macroglobulinemia.[3] Types II and III are strongly associated with infection by the hepatitis C virus.[3] There are three different types of cryoglobulins that have been observed to form in the blood.[4] Description These are composed of a single monoclonal immunoglobulin paraprotein (usually IgM). 10-15% of Type isolated monoclonal Sometimes, these are represented by light chains only the total I immunoglobulins and can be extracted from the urine, or they will cases accumulate in blood serum in the event of renal failure.[5] They usually have a polyclonal component, usually IgG, and a monoclonal component, usually IgM, immunocomplexes 50-60% of Type which has an RF function. The IgM can recognize formed by monoclonal reported II intact IgG or either the Fab region or Fc region of IgG IgM cases fragments. This is why most type II cryoglobulins are IgM-IgG complexes.[5] 25-30% of immunocomplexes These have very similar function to the type II Type the formed by polyclonal cryoglobulins, however they are composed of III reported IgM polyclonal IgM and IgG molecules.[5] cases 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.[5] 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[6] Type Composition Percent

[edit] 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 occasionally as a symptom in 35% of chronic hepatitis C infections.[7] 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)[8] is generally seen with polyclonal CGs seen in essential-, viral-, or connective tissue disease-associated CG.

[edit] See also

Cryoglobulinemic purpura Hematopoietic ulcer Paraproteinemia

[edit] References
1. 2. ^ "cryoglobulinemia" at Dorland's Medical Dictionary ^ 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): 77588. 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): 413. doi:10.1136/jcp.55.1.4. PMC 1769573. PMID 11825916. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=1769573. ^ "cryoglobulin" at Dorland's Medical Dictionary ^ a b c d Tedeschi A, Barat C, Minola E, Morra E (2007). "Cryoglobulinemia". Blood Rev. 21 (4): 183200. doi:10.1016/j.blre.2006.12.002. PMID 17289231. http://linkinghub.elsevier.com/retrieve/pii/S0268960X(06)00076-2. ^ 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): 6375. doi:10.1016/0022-1759(94)90284-4. PMID 8034987. ^ Pascual M, Perrin L, Giostra E, Schifferli JA (1990). "Hepatitis C virus in patients with cryoglobulinemia type II". J. Infect. Dis. 162 (2): 56970. doi:10.1093/infdis/162.2.569. PMID 2115556. ^ 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): 11526. PMID 7704562. http://qjmed.oxfordjournals.org/cgi/pmidlookup?view=long&pmid=7704562.

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4. 5.

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7. 8.

[edit] External links

Overview at Mayo Clinic

[hide]v d eImmune disorders, Immunoproliferative immunoglobulin disorders

(D89, 273)
Plasmacytoma Multiple myeloma (Plasma cell leukemia) MGUS IgM (Macroglobulinemia/Waldenstrm's macroglobulinemia) heavy chain (Heavy chain disease) light chain (Primary amyloidosis) proc, drug(L3/4)

PCDs/PP

Other hypergammaglobulinemia Cryoglobulinemia M: LMC cell/phys/auag/auab/comp, imdf/ipig/hyps/tumr igrc

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