Cryoglobulinemia

Causes, incidence, and risk factors

Cryoglobulins are antibodies . It is not yet known why they become solid or gel-like at low temperatures. When this occurs, these antibodies can block blood vessels. This may lead to problems ranging from skin rashes to kidney failure.

Cryoglobulinemia is part of a group of diseases that cause damage and inflammation of the blood vessels throughout the body (vasculitis). There are three main types of the disorder. They are grouped based on the type of antibody that is produced:

• Cryoglobulinemia type I
• Cryoglobulinemia typeII
• Cryoglobulinemia type III

Types II and III are also referred to as mixed cryoglobulinemia.

Type I cryoglobulinemia is most often related to cancer of the blood or immune systems.

Types II and III are most often found in people who have a chronic (long-lasting) inflammatory condition, such as an autoimmune disease or hepatitis C. Most people with mixed cryoglobulinemia have a chronic hepatitis C infection.

Other conditions that may be related to cryoglobulinemia include:

• Leukemia
• Multiple myeloma
• Mycoplasma pneumonia
• Primary macroglobulinemia
• Rheumatoid arthritis
• Systemic lupus erythematosus

Symptoms

Symptoms will vary depending on the type of disorder you have and the organs that are involved. Symptoms may include:

• Breathing problems
• Fatigue
• Glomerulonephritis
• Joint pain
• Muscle pain
• Purpura
• Raynaud's phenomenon
• Skin death
• Skin ulcers

Signs and tests

The doctor will do a physical exam.You will be checked for signs of liver and spleen swelling.

Tests for cryoglobulinemia include:

• Complete blood count (CBC)
• Complement assay -- numbers will be low
• Cryoglobulin test -- may show presence of cryoglobulins
• Liver function tests -- may be high
• Rheumatoid factor -- positive in types II and III
• Skin biopsy
• Urinalysis -- may show blood in the urine if the kidneys are affected

Other tests may include:

• Angiogram
• Chest x-ray
• ESR
• Hepatitis C test
• Nerve conduction tests, if the person has weakness in the arms or legs
• Protein electrophoresis - blood

Treatment

Mild or moderate forms of cryoglobulinemia can often be treated by taking steps to deal with the underlying cause.  

• Mild cases can be treated by avoiding cold temperatures.
• Standard hepatitis C treatments usually work for patients who have hepetitis C and mild or moderate disease. The condition can return when treatment stops.
  

Severe cryoglobulinemia involves vital organs or large areas of skin. It is treated with corticosteroids and other medicines that calm the immune system.

Treatment may also involve plasmapheresis. In this procedure, blood plasma is taken out of blood circulation. It is replaced by fluid, protein, or donated plasma.

Expectations (prognosis)

Cryoglobulinemia is not usually deadly. However, if the kidneys are affected, the outlook is poor.

Complications

Complications include:

• Bleeding in the digestive tract (rare)
• Heart disease (rare)
• Infections of ulcers
• Kidney failure
• Liver failure
• Skin death
• Death

Calling your health care provider

Call your health care provider if:

• You develop symptoms of cryoglobulinemia
• You have hepatitis C and develop symptoms of cryoglobulinemia
• You have cryoglobulinemia and develop new or worsening symptoms

Prevention

There is no known prevention. Avoiding exposure to cold temperatures may prevent some symptoms.

Because so many cases of mixed cryoglobulinemia are associated with hepatitis C, prevention of hepatitis C infection may reduce your risk of cryoglobulinemia.

References

Goldman L, Ausiello D. Cecil Textbook of Medicine. 22nd ed. Philadelphia, Pa: WB Saunders; 2004:1193.

Rakel P, ed. Conn’s Current Therapy 2006. 58th ed. Philadelphia, Pa: WB Saunders; 2006:980, 1164.

Harris ED, Budd RC, Genovese MC, Firestein GS, Sargent JS, Sledge CB. Kelley's Textbook of Rheumatology. 7th ed. St. Louis, Mo: WB Saunders; 2005:1392-1393.