Cryoglobulinemia – essential mixed
Overview
Cryoglobulinemia is a medical condition that is caused by proteins called cryoglobulins, which are present in the blood. Cryoglobulins are abnormal proteins that by definition have the unusual property of precipitating from the serum specimen when it is chilled in the laboratory 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.
Causes
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.
Prognosis
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
Treatment
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.