Cryoglobulinemia

Cryoglobulinemia is the presence of abnormal proteins that are occasionally found in the blood of people with some forms of autoimmune diseases, multiple myeloma, leukemia, and certain forms of pneumonia. The proteins cause the blood to gel at low temperatures causing tissue necrosis.

Causes:  Type 1 cryoglobulinemia is often associated with lymphoma.  Type 2 cryoglobulinemia is often associated with hepatitis C infection.  Drug usage is a prime risk factor for patients with cryoglobulinemia.  Hepatitis C is acquired by injection drug use (needle-sharing), tainted blood products, and (probably rarely), sexual transmission.

Symptoms may include a rash on the lower limbs, arthritis, nerve damage and tissue necrosis of affected areas.

Treatment depends on the type of cryoglobulin, underlying disease, and severity of symptoms. Cryoglobulinemia with severe hyperviscosity syndrome requires plasmapheresis and chemotherapy of the underlying malignancy. Some patients with cryoglobulinemia suffer from mild, recurrent crops of lower extremity purpura that require no specific therapy. The most effective treatment for cryoglobulinemia associated with hepatitis C has not yet been determined. Treating the hepatitis may be an effective therapy for this type of vasculitis. Brief use of prednisone followed by 6 months of interferon alfa has produced clinical and liver function test improvement, but relapse of liver disease and vasculitis often occurs when interferon alfa is stopped.

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