ESSENTIAL MIXED CRYOGLOBULINEMIA

ESSENTIAL MIXED CRYOGLOBULINEMIA

Essential mixed cryoglobulinemia is one of the subdivisions of mixed cryoglobulinemia. It occurs when there is a mixture of different types of antibodies, forming for unknown reasons. 

Cryoglobulins are abnormal blood proteins with unusual properties precipitating from the blood serum when chilled and redissolving upon rewarming. They can cause problems as the blood goes abnormally thick, increasing the risk of blood clots forming in the brain, eyes, and heart. 

The disease is rare and often associated with Hepatitis C virus infection. Since its association, lesser people have been diagnosed with essential cryoglobulinemia.

TYPES 

Mixed cryoglobulinemia is synonymous with Cryoglobulinemic Vasculitis. Here is the subdivision of mixed cryoglobulinemia;

  • Essential Mixed Cryoglobulinemia
  • Type I Cryoglobulinemia
  • Type II Cryoglobulinemia
  • Type III Cryoglobulinemia

 

SYMPTOMS

Common symptoms may include:

  • Skin lesions 
  • Joint pain and arthritis
  • Enlargement of the spleen 
  • Nerve and kidney disease

 

However, specific symptoms may vary from one person to another and can involve other organ systems. 

  • Skin vasculitis with purplish patches is another symptom associated with cryoglobulinemia. Individuals affected develop purplish discoloration of the skin. It is due to the bleeding underneath the skin coming from the small blood vessels. 

In some cases, cryoglobulinemia is associated with membranoproliferative glomerulonephritis (MPGN) type I, a renal kidney disease. The illness is described as having a specific injury pattern in the kidney caused by the deposition of cryoglobulins present in the kidney. This type of kidney disease can be a severe complication leading to kidney failure. 

Chronic hepatitis can be associated with essential mixed cryoglobulinemia, specifically hepatitis C. Both hepatitis C and cryoglobulinemia have similar symptoms like fatigue and aching joints and muscles. Eventually, hepatitis can progress to scarring of the liver.

It can lead to recurrent pain in the abdomen, heart attack, and bleeding in the lungs. 

Weight loss and poor appetite can also be a symptom.

DIAGNOSIS

The diagnosis process may be conducted upon identification of characteristic symptoms. The doctor will look into detailed patient history, a thorough clinical evaluation, and a blood test to detect cryoglobulins’ presence.

  • A blood test is a crucial test for anyone with mixed cryoglobulinemia. The blood sample has to be in 37 Celcius, and it should remain at its temperature until it is placed in a room temperature centrifuge. Usually, the blood sample is required to be stored for 5-7 days.

A viral and antibody test may be performed by your doctor to detect rheumatoid factor. The test may be positive in 80 to 90 percent of cases. 

 

TREATMENT

This rare condition can be treated with medications that reduce inflammation and suppress the immune system.

Here are the medications used to treat essential mixed cryoglobulinemia: 

  • Non-steroid anti-inflammatory drugs (ibuprofen, aspirin); 
  • Steroids (prednisone, prednisolone), cyclophosphamide (Cytoxan), chlorambucil (Leukeran); and, 
  • Azathioprine (Imuran). 

For severe symptoms, plasmapheresis is performed. The said procedure will require the blood’s serum to be filtered to remove the cryoglobulins. 

Doctors consider using interferon-alpha for patients with the hepatitis C virus, especially those with mild disease or remission of manifestations after immunosuppressive treatment.

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