Cryoglobulinemia pathophysiology: Difference between revisions
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==Pathophysiology== | ==Pathophysiology== | ||
Cryoglobulins are proteins (single or mixed immunoglobulins) that precipitate from serum and plasma when cooled. They are produced due to chronic immune system activation and lypmphoproliferation. Cryoglobulins have a tendency to redissolve on rewarming. The pathogenesis of cryoglobulinemia differs slightly based on the type of disorder and disease associations. The following are the major mechanisms involved in the pathogenesis of cryoglobulinemia: | |||
It is important to note that these two different, yet highly representative, clinical syndromes generally reflect different types of underlying CG: | It is important to note that these two different, yet highly representative, clinical syndromes generally reflect different types of underlying CG: | ||
Revision as of 13:46, 1 May 2018
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor-In-Chief: Cafer Zorkun, M.D., Ph.D. [2]
Overview
Pathophysiology
Cryoglobulins are proteins (single or mixed immunoglobulins) that precipitate from serum and plasma when cooled. They are produced due to chronic immune system activation and lypmphoproliferation. Cryoglobulins have a tendency to redissolve on rewarming. The pathogenesis of cryoglobulinemia differs slightly based on the type of disorder and disease associations. The following are the major mechanisms involved in the pathogenesis of cryoglobulinemia:
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" of palpable purpura, arthralgia and myalgia is generally seen with polyclonal CGs seen in essential-, viral-, or connective tissue disease-associated CG.
- MC is closely associated with hepatitis C infection and is thought to activate B lymphocytes by binding to CD81.
- 80-95% of patients with MC have circulating anti-HCV antibodies or circulating HCV RNA in the serum or within the cryoprecipitate.
- Polyclonal IgG anti-HCV have been noted in the cryoprecipitate as well.
- Approximately 50% of patients with chronic hepatitis C and 15% with hepatitis B will have circulating MC (1/2 Type II, 2/3 Type III).
- It is unclear what the antigen trigger is for production of the MC, but it is though that the hepatitis C viral RNA itself may be the factor since it is found in high quantities in the cryoprecipitate.