About essential mixed cryoglobulinemia

What is essential mixed 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 when it is chilled in the laboratory and then dissolves back into the serum upon rewarming.

Cryoglobulins may or may not cause 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 a variety of problems throughout the body. These include complications 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 blood flow 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 are the symptoms for essential mixed cryoglobulinemia?

Enlargement of lymph nodes symptom was found in the essential mixed cryoglobulinemia condition

Cryoglobulinemia has been associated with:

  • Infections. Hepatitis C is the most common infection associated with cryoglobulinemia. Others include hepatitis B, HIV, Epstein-Barr, toxoplasmosis and malaria.
  • Certain cancers. Some cancers of the blood, such as multiple myeloma, Waldenstrom macroglobulinemia and chronic lymphocytic leukemia, can sometimes cause cryoglobulinemia.
  • Autoimmune disorders. Disease such as lupus, rheumatoid Arthritis and Sjogren's syndrome increase the risk of developing cryoglobulinemia.

What are the causes for essential mixed cryoglobulinemia?

Cryoglobulinemia has been associated with:

  • Infections. Hepatitis C is the most common infection associated with cryoglobulinemia. Others include hepatitis B, HIV, Epstein-Barr, toxoplasmosis and malaria.
  • Certain cancers. Some cancers of the blood, such as multiple myeloma, Waldenstrom macroglobulinemia and chronic lymphocytic leukemia, can sometimes cause cryoglobulinemia.
  • Autoimmune disorders. Disease such as lupus, rheumatoid arthritis and Sjogren's syndrome increase the risk of developing cryoglobulinemia.

What are the treatments for essential mixed cryoglobulinemia?

The treatment of mixed cryoglobulinemia may require the coordinated efforts of a team of specialists. Pediatricians, dermatologists, neurologists, kidney specialists (nephrologists), liver specialists (hepatologists), specialists in diagnosing and treating blood disorders (hematologists), and other healthcare professionals may need to systematically and comprehensively plan an affect individual’s treatment.

Initial treatment is directed against the underlying condition associated with the disorder, which in most cases will be infection with the hepatitis C virus. Affected individuals are treated with pegylated interferon usually in conjunction with ribavirin to eradicate the hepatitis C virus. This treatment often leads to significant clinical improvement.

Additional therapies are tailored to the individual patient based upon numerous factors including the severity and progression of the disease, specific organ involvement, any associated disorders, overall health, patient preference, and additional factors. These therapies may be used alone or in conjunction with one another.

Immunosuppressive drugs such as corticosteroids, cyclophosphamide, or azathioprine may be used in individuals with organ involvement including vasculitis, kidney disease, neurological symptoms, or disabling skin abnormalities. These drugs require careful monitoring because they can cause virus replication. Immunosuppressive drugs may be the initial therapy in individuals without a known underlying cause to their cryoglobulinemia (essential mixed cryoglobulinemia).

A newer immunosuppressive drug known as rituximab has been increasingly used for the major complications associated with mixed cryoglobulinemia (see Investigational Therapies below).

Additional treatment is symptomatic and can include non-steroidal anti-inflammatories drugs (NSAIDs) for the treatment of fatigue and arthralgia. Some individuals may be treated with a low antigen complement (LAC) diet. This diet is designed to promote the clearance of circulating immune complexes (cryoglobulins) from the blood. The LAC diet is best for individuals with mild mixed cryoglobulinemia and often used in conjunction with low doses of corticosteroids.

Investigational Therapies

Researchers are studying the use of the immunosuppressive drug rituximab, which targets specific white blood cells known as B-lymphocytes, for the treatment of individuals with mixed cryoglobulinemia. Rituximab is classified as a monoclonal antibody or biologic therapy – medications that act like antibodies, but are artificially created in a lab. Initial studies have shown that the drug is safe and effective. Advantages of rituximab are that it avoids serious immunosuppression and side effects associated with other immunosuppressive agents. More research is necessary to determine the long-term safety and effectiveness of rituximab for the treatment of mixed cryoglobulinemia.

Some individuals who have severe mixed cryoglobulinemia may undergo a procedure known as plasmapheresis. These procedures are usually reserved for individuals who have not responded to other treatment options and are experiencing severe or life-threatening complications. Plasmapheresis involves removing unwanted substances (e.g. cryoglobulins) from the blood. Blood is removed from the patient and the solid blood cells are separated from the liquid plasma. The patient’s plasma is then replaced with donor human plasma or albumin, which is re-transfused, along with the patient’s original blood cells.

What are the risk factors for essential mixed cryoglobulinemia?

Risk factors of cryoglobulinemia may include:

  • Your sex. Cryoglobulinemia occurs more frequently in women than in men.
  • Age. Symptoms of cryoglobulinemia usually begin in middle age.
  • Other diseases. Cryoglobulinemia is associated with diseases such as hepatitis C, HIV, multiple myeloma, Waldenstrom macroglobulinemia, lupus and Sjogren's syndrome.

Is there a cure/medications for essential mixed cryoglobulinemia?

The coordinated efforts of a group of experts may be necessary for the treatment of mixed cryoglobulinemia. It may be necessary for pediatricians, dermatologists, neurologists, nephrologists, hepatologists, specialists in the diagnosis and treatment of blood problems (hematologists), and other medical professionals to systematically and thoroughly organize a patient's care.

  • The primary goal of treatment is to address the disorder's underlying disease, which is typically hepatitis C virus infection. Pegylated interferon is typically used in conjunction with ribavirin to treat affected people and get rid of the hepatitis C virus.
  • A considerable clinical improvement is frequently seen after this treatment.
  • Additional treatments are personalized to each patient based on a variety of characteristics, such as the disease's severity and course, the involvement of particular organs, any accompanying illnesses, general health, patient preferences, and other factors.
  • These treatments can be used separately or in combination.
  • Non-steroidal anti-inflammatories medications (NSAIDs) for the treatment of fatigue and arthralgia are examples of further symptomatic treatment.
  • A diet known as a low antigen complement (LAC) diet may be used to treat some patients.
  • This diet is intended to encourage the removal of blood-borne immunological complexes (cryoglobulins) from circulation.
  • The LAC diet is frequently used with low doses of corticosteroids and is most effective for people with mild mixed cryoglobulinemia.

Joint pain,Arthritis,Enlargement of lymph nodes
Discoloration of skin,Bruising

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