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Cryoglobulinemia

 

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

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 cause inflammation and 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 cryoglobulinemia. They are grouped based on the type of antibody that is produced:

  • Type I
  • Type II
  • 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 this form of the disease 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 health care provider 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 (It is a good idea to check to make sure the lab has experience drawing and storing blood for this test.)
  • Liver function tests -- may be high if hepatitis C is present
  • Rheumatoid factor -- positive in types II and III
  • Skin biopsy -- may show inflammation in blood vessels, vasculitis
  • Protein electrophoresis - blood -- may show an abnormal antibody protein
  • 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

 

Treatment

 

Mixed cryoglobulinemia (Types II and III)

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 treatments work for most people who have hepatitis C and mild or moderate disease. The condition can come back when treatment stops.

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

  • Rituximab is an effective drug and has fewer risks than other medicines.
  • Cyclophosphamide is used in life-threatening conditions where rituximab is not working or available. This medicine was used often in the past.
  • A treatment called plasmaphereis is also used. In this his procedure, blood plasma is taken out of blood circulation and abnormal cryoglobulin antibody proteins are removed. The plasma is replaced by fluid, protein, or donated plasma.

Type I cryoglobulinemia:

This disorder is due to a cancer of the blood or immune system such as multiple myeloma . Treatment is directed against the abnormal cancer cells that produce the cryoglobulin.

 

Outlook (Prognosis)

 

Most of the time, mixed cryoglobulinemia does not lead to death. 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 for the condition.

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

 

 

Open References

References

Dammacco F, Sansonno D. Therapy for hepatitis C virus-related cryoglobulinemic vasculitis. N Engl J Med . 2013;369:1035. PMID: 24024840 www.ncbi.nlm.nih.gov/pubmed/24024840 .

De Vita S, Quartuccio L, Isola M, et al. A randomized controlled trial of rituximab for the treatment of severe cryoglobulinemic vasculitis. Arthritis Rheum . 2012;64:843. PMID: 22147661 www.ncbi.nlm.nih.gov/pubmed/22147661 .

Rajkumar VS. Plasma cell disorders. In: Goldman L, Schafer AI, eds. Goldman's Cecil Medicine . 24th ed. Philadelphia, PA: Elsevier Saunders; 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: Elsevier Saunders; 2012:chap 91.

 
  • Cryoglobulinemia - of the fingers

    Cryoglobulinemia - of the fingers - illustration

    Cryoglobulinemia is caused by an abnormal protein that is occasionally found in the blood of people with multiple myeloma, leukemia, and certain forms of pneumonia. It causes blood to gel at low temperatures. In this picture, cryoglobulinemia has reduced blood flow in the fingers so much the fingers have turned dark; the black areas are gangrene resulting from lack of blood flow.

    Cryoglobulinemia - of the fingers

    illustration

  • Cryoglobulinemia - fingers

    Cryoglobulinemia - fingers - illustration

    Tissue necrosis caused by distal capillary thrombosis resulting from cryoglobulin precipitation in the vessels. The black tissue under the nails is ischemic, and will eventually slough, or be reabsorbed by the body.

    Cryoglobulinemia - fingers

    illustration

  • Blood cells

    Blood cells - illustration

    Blood is comprised of red blood cells, platelets, and various white blood cells.

    Blood cells

    illustration

    • Cryoglobulinemia - of the fingers

      Cryoglobulinemia - of the fingers - illustration

      Cryoglobulinemia is caused by an abnormal protein that is occasionally found in the blood of people with multiple myeloma, leukemia, and certain forms of pneumonia. It causes blood to gel at low temperatures. In this picture, cryoglobulinemia has reduced blood flow in the fingers so much the fingers have turned dark; the black areas are gangrene resulting from lack of blood flow.

      Cryoglobulinemia - of the fingers

      illustration

    • Cryoglobulinemia - fingers

      Cryoglobulinemia - fingers - illustration

      Tissue necrosis caused by distal capillary thrombosis resulting from cryoglobulin precipitation in the vessels. The black tissue under the nails is ischemic, and will eventually slough, or be reabsorbed by the body.

      Cryoglobulinemia - fingers

      illustration

    • Blood cells

      Blood cells - illustration

      Blood is comprised of red blood cells, platelets, and various white blood cells.

      Blood cells

      illustration

    Tests for Cryoglobulinemia

     
       

      Review Date: 1/20/2015

      Reviewed By: Gordon A. Starkebaum, MD, Professor of Medicine, Division of Rheumatology, University of Washington School of Medicine, Seattle, WA. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

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