Cryoglobulinemia pathophysiology
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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
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.