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CRYOGLOBULINEMIA AND CRYOFIBRINOGENEMIA
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Cryoglobulinemia (CG) and cryofibrinogenemia (CF) lead to clinical manifestations through the formation of cryoprecipitates. Dermatologic manifestations can be secondary to hyperviscosity or cold-induced thrombosis (type I CG and CF), or immune-complex mediated phenomena (types II & III, ie, mixed CGs). All patients should receive appropriate supportive care (analgesia, wound care, cold avoidance) and treatment for any identifiable underlying cause. Management of the associated underlying disease is critical to controlling CG /CF. This typically requires coordination with additional specialists to address the underlying cause (most often hematology, rheumatology, hepatology, or infectious diseases); treatment will lead to resolution of the CG/CF. For example underlying Hepatitis C should be treated promptly. Current guidelines recommend patients with mixed CG secondary to hepatitis C receive identical antiviral therapy as those without associated CG.25 For mixed CG associated with HIV or hepatitis B infection, antiviral therapy should precede or coincide with immunosuppressive therapy due to increased risk of viral replication following immunosuppression. For other etiologies, immunosuppression should precede or coincide with treatment of the underlying cause.
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Adjunctive treatments may be necessary: patients with mild disease do not typically require additional treatment, but if needed non-ulcerating skin lesions in those with mixed CG vasculitis can be managed as idiopathic cutaneous small vessel vasculitis. Patients with more severe manifestations typically require additional treatments including immunosuppression (CG and CF), plasmapheresis (CG and CF), or fibrinolytics (CF). The table below outlines adjunctive treatments that can be considered. Patients receiving immunosuppressive medications should receive prophylaxis against opportunistic infections and immunizations as appropriate.1,2 See Table 131-1.
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