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Cryoglobulinemia
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Cryoglobulinemia

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Cryoglobulinemia is the presence of abnormal proteins in the blood. These proteins thicken in cold temperatures.

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 type II
  • 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

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  • Causes

    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 type II
    • 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
  • Exams 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.

  • Outlook (Prognosis)

    Most of the time cryoglobulinemia is not usually deadly. Outlook can be poor if the kidneys are affected.

  • Possible Complications

    Complications include:

    • Bleeding in the digestive tract (rare)
    • Heart disease (rare)
    • Infections of ulcers
    • Kidney failure
    • Liver failure
    • Skin death
    • Death
  • When to Contact a Medical Professional

    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.

    • Staying away from cold temperatures may prevent some symptoms.
    • Testing and treatmentment for hepatitis C infection may reduce your risk of the condition.

Related Information

  Protein in dietMycoplasma pneumon...Autoimmune disorde...Systemic lupus ery...Rheumatoid arthrit...Cryoglobulins     PneumoniaSystemic lupus ery...Rheumatoid arthrit...

References

Rajkumar VS. Plasma Cell Disorders. In: Goldman L, Schafer AI, eds. Cecil Medicine. 24th ed. Philadelphia, Pa: Saunders Elsevier; 2011:chap 193.

Stone JH. Immune Complex-Mediated Small Vessel Vasculitis. In: Firestein GS, Budd RC, Gabriel SE, et al, eds. Kelley's Textbook of Rheumatology. 9th ed. Philadelphia, Pa: Saunders Elsevier; 2012:chap 91.

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Review Date: 1/22/2013  

Reviewed By: Todd Gersten, MD, Hematology/Oncology, Palm Beach Cancer Institute, West Palm Beach, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA and the A.D.A.M. Health Solutions, Ebix, Inc., Editorial Team.

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