What is Cryoglobulinemia?
http://www.mayo.edu/mmgrg/rst/cryo.htm#1
Cryoglobulins are proteins that precipitate (or clump together) in the cold. For
most people this may be solely an abnormal laboratory finding, and they will
have no symptoms because of their cryoglobulins. For others, however, these
cryoglobulins can cause disease that affects any or all organs. When there are
symptoms secondary to the cryoglobulins, the disease is called
"Cryoglobulinemia."
The proteins that comprise cryoglobulins are called antibodies or
immunoglobulins. The normal role for these immunoglobulins or antibodies is to
help the body fight infection. Unfortunately, in the disease
cryoglobulinemia, these immunoglobulins may clump together, causing organ damage
and illness.
Cryoglobulinemia may or may not be associated with other diseases (like liver
disease, infection, a connective tissue disease, multiple myeloma, or lymphoma).
What are the symptoms of cryoglobulinemia?
Cryoglobulinemia is a syndrome comprised of many possible symptoms. The most
common symptoms are those of weakness, arthralgia (joint ache), and
purpura (purple bruises, most commonly of the lower extremities). Besides the
skin and joints, the other organs commonly involved include nerves, kidneys, and
liver. Symptoms and signs may include:
Weakness
Joint aches (arthralgias)
Purple/red spots on the extremities
Raynaud's phenomenon
Fever
Weight loss
High blood pressure
Swelling of ankles and legs
Numbness, tingling, or weakness in the hands or feet
Skin ulcers and gangrene
Enlarged liver or spleen
Abdominal pain
Kidney damage
The course of the disease will vary. Symptoms may come and go seemingly without
explanation. For some, but not all, cold temperatures will cause
flare-ups.
Who is affected by cryoglobulinemia?
Cryoglobulinemia is very rare. Its cause is not known, but there is an
association with certain diseases as well as the hepatitis C virus. It can
affect anyone, but the majority of people with cryoglobulinemia are
in their 40s to 50s. Twice as many women as men have this disorder.
Cryoglobulinemia is not inherited. It is not known how many people have
this disease.
How is cryoglobulinemia diagnosed?
The diagnosis is made by combining the information from a thorough history and
physical examination and from blood and urine tests. At times, biopsies (small
samples of tissue) are also required. Potential biopsy sites include the skin,
kidney, bone marrow, liver, and a peripheral nerve.
How is cryoglobulinemia treated?
There are few standard treatments for symptomatic cryoglobulinemia. Important
questions before deciding upon treatment options include the
following: 1) Am I having symptoms related to my cryoglobulins? 2) How severe
are my symptoms and which of my organs appear to be affected? 3) Do I have an
underlying disease related to my cryoglobulinemia? 4) Am I infected with the
hepatitis C virus?
The goal of treatment is to limit further organ damage. Because cryoglobulinemia
is so rare, very few clinical trials have been completed to address questions
about the best treatment strategies. In
general, all patients should: avoid the cold, if it seems to aggravate their
symptoms; consume an adequate well-balanced diet; and maintain their strength
with adequate rest and exercise. You may need to follow a
special diet based on your symptoms and affected organs. For example, if your
kidneys are affected, you may be asked to follow a low salt diet.
The typical treatment strategy is to treat those patients with cryoglobulinemia
"secondary" to another disease with the preferred treatment for that particular
disease. For example, if you have multiple myeloma and cryoglobulinemia, your
doctor (hematologist/oncologist) should use the standard treatment strategies
for patients with multiple
myeloma. If you have cryoglobulins secondary to rheumatoid arthritis, your
doctor (rheumatologist) should use the drugs used to treat rheumatoid arthritis.
In contrast, if you have "primary" or "essential" cryoglobulinemia, your doctor
must look very hard for the presence of a hepatitis virus. If there is hepatitis
virus, a medicine called interferon-alpha may be recommended. This medicine has
a number of side effects, but can control the hepatitis C and the symptoms of
cryoglobulinemia for a proportion of
patients. Unfortunately, not all patients will tolerate this medication; others
will not derive any benefit; and still others will relapse once the medication
has been discontinued.
Your doctor may advise no therapy, but rather only careful monitoring.
Alternatively, s/he may prescribe corticosteroids (prednisone),
anti-inflammatory medicines, immunosuppressive medicines and sometimes low-dose
chemotherapy to control the symptoms. If there is a severe illness related to
the cryoglobulinemia, your doctor may suggest a
procedure called plasmapheresis, whereby your blood plasma (which contains much
of your cryoglobulins) is exchanged for either someone else's plasma or some
other type of replacement fluid.
Does cryoglobulinemia make me more susceptible to infections?
You may experience more infections. Whether the infections are related to the
cryoglobulinemia directly or to many of the treatments doctors use to control
the symptoms of cryoglobulinemia is unclear. If possible, you should avoid
direct exposure to people with known viral or bacterial infections (i.e. cold or
flu).
Dealing with the stress of cryoglobulinemia
Cryoglobulinemia can be a stressful disease. You may worry about how the disease
will affect you and your family. While these fears and worries are normal, they
may not always be understood by relatives or friends. Talking with a physician,
nurse or other health professional may help you deal with these fears. The more
cryoglobulinemia is understood, the less frightening it is.
Who can I call if I have further questions?
If you have any further questions about your diagnosis or treatment, call the
Mayo Clinic location where you are a patient.
Mayo Clinic Jacksonville
(904) 953-2000 (ask for your physician)
Mayo Clinic Rochester
(507) 284-2511 (ask for your physician)
Mayo Clinic Scottsdale
(602) 301-8000 (ask for your physician)
This material does not provide all available information about Cryoglobulinemia
and is not intended as a substitute for seeking
professional medical care and advice.
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