Essential Mixed Cryoglobulinemia
Medical Author: William C. Shiel Jr., MD, FACP, FACR
What is cryoglobulinemia?
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.
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.
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Last Editorial Review: 11/7/2007
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