Cryoglobulinemia

What is cryoglobulinemia?

Cryoglobulins are proteins found in the blood that precipitate (clump together) in the cold and may cause inflammation and organ damage. However, these proteins can also be present in low levels in the blood without causing any symptoms. When there are symptoms due to the cryoglobulins, the disease (usually a special rash) is called "cryoglobulinemia."

What causes cryoglobulinemia?

Cryoglobulinemia can be present alone ("idiopathic") or can be associated with other diseases such as:

• Infection, particularly hepatitis C infection.
• Blood cell abnormalities such as lymphoma and multiple myeloma.
• Connective tissue disease such as lupus.

What are the symptoms of cryoglobulinemia?

Most people with cryoglobulins have no symptoms other than elevated levels on specially ordered lab tests. When symptoms are present, they are most commonly fatigue, joint pain, numbness or weakness, and a particular rash called purpura that looks like red spots or purple bruises, usually over the lower legs.

Other symptoms and signs may include:

• Change of the color of hands and/or feet (from normal to white to a purplish-blue color) with cold, called "Raynaud's Phenomenon," but Raynaud's is very common in persons without cryoglobulinemia.
• Weight loss.
• High blood pressure.
• Swelling of ankles and legs.
• Skin ulcers and gangrene.
• Enlarged liver or spleen.
• Numbness, tingling or weakness of hands or feet.
• Kidney damage.

How is cryoglobulinemia diagnosed?

Cryoglobulinemia is diagnosed by a specific blood test that detects the presence of cryoglobulins in the blood. Learning the type of cryoglobulins can sometimes help determine its cause. It takes almost a week after the test is ordered to get the result.

How is cryoglobulinemia treated?

Treatment of cryoglobulinemia is not always necessary and depends on the organs affected, degree of damage, and presence of other medical conditions. It is very important not only to treat the cryoglobulinemia but also to address any other associated disorders.

The mainstay of treatment is corticosteroids with or without other medications depending on the affected organ and the extent of involvement.

Another form of treatment decreases the amount of cryoglobulins in the blood. This procedure, called plasmapheresis, removes cryoglobulins from the plasma (the liquid in the blood). This helps prevent cryoglobulins from clogging the arteries, which blocks blood flow and could lead to the rash and organ damage.

If another medical condition has been found, such as hepatitis C, anti-viral therapy may be recommended, often with referral to a hepatologist (liver specialist).

If multiple myeloma or lymphoma is found, a referral to a hematologist is recommended.