Essential Mixed Cryoglobulinemia
What is cryoglobulinemia?
Cryoglobulinemia is a medical condition that is caused by proteins called cryoglobulins present in the blood. Cryoglobulins are abnormal proteins that by definition have the unusual property of precipitating from the serum specimen in the laboratory when it is chilled and redissolving into the serum upon rewarming.
Cryoglobulins may or may not be causing disease. Cryoglobulins can accompany another condition (such as dermatomyositis, multiple myeloma, or lymphoma) or be an isolated condition themselves, called cryoglobulinemia.
What conditions are associated with cryoglobulinemia?
Cryoglobulins in the blood (cryoglobulinemia) can cause conditions throughout the body. These conditions include problems resulting from abnormal "thickness" of the blood (such as stroke or blood clots in the eyes leading to blindness) and inflammation of blood vessels, referred to as vasculitis. Vasculitis of arteries can result in blockage of arteries leading to damage to the organ(s) supplied by the affected blood vessels, such as in the skin, kidneys, or elsewhere. What is essential mixed cryoglobulinemia?
When the cryoglobulin proteins are a mixture of various antibody types, and forming for unknown reasons (essential), the conditions is referred to as essential mixed cryoglobulinemia.
Essential mixed cryoglobulinemia is characterized by joint pains and swelling (arthritis), enlargement of the spleen, skin vasculitis with purplish patches, and nerve and kidney disease. This can lead to recurrent pain in the abdomen, heart attack, and bleeding in the lungs. Weight loss can occur as well as poor appetite.
Essential mixed cryoglobulinemia is sometimes associated with hepatitis C virus infection.
What is the prognosis (outlook) for patients with essential mixed cryoglobulinemia?
The prognosis and natural history of the illness is not predictable. Kidney damage can be serious and recent reports state that permanent failure of the kidney occurs in approximately 10 % of patients. Death can occur, usually from serious heart disease, infection, or brain hemorrhage.
How is essential mixed cryoglobulinemia treated?
Essential mixed cryoglobulinemia is treated with combinations of medications which reduce inflammation and suppress the immune system. Medications used include nonsteroid antiinflammatory drugs (ibuprofen, aspirin, and others), cortisone preparations (prednisone, prednisolone), cyclophosphamide (Cytoxan), chlorambucil (Leukeran), and azathioprine (Imuran). Plasmapheresis (hemapheresis), a procedure whereby the blood's serum is replaced with saline (salt water), is also performed for severe symptoms.
Recent studies have demonstrated some benefit of using interferon- alpha for those patients with evidence of hepatitis C virus, particularly those with mild disease or in those with remission of manifestations after immune suppression treatment.
Author: William C. Shiel Jr., MD, FACP, FACR
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