Cryoglobulinemia

Medical quality assurance by Dr. Albrecht Nonnenmacher, MD at August 4, 2016
StartDiseasesCryoglobulinemia

Cryoglobulinemia, pronounced "kry-oh-glob-u-lih-NE-me-uh," is a condition where blood contains abnormal amounts of certain proteins (antibodies) and which results in adverse and severe health effects. Cryoglobulinemia affects the blood plasma, which comprises more than half of the content of human blood. Cryoglobulinemia continues to confound researchers regarding how it arises and progresses.

Contents

Definition & Facts

Cryoglobulinemia primarily affects adults that are over the age of 50. It is considered to be one of several diseases grouped together under the umbrella of vasculitis, which describes inflammation or damage of the blood vessels in the body. Cryoglobulins are abnormal blood proteins.

The word "cryoglobulinemia" can be separated out into "cryoglobulin" and "emia." Cryoglobulins are irregular blood proteins. The word "cryoglobulinemia" itself translates to mean "cold antibodies in the blood."  The coldness refers to the tendency of cryoglobulins to solidify at lower temperatures. In more extreme cases of the condition, this can cause the blood itself to become thick and syrupy (one analogy used is maple syrup).

Symptoms & Complaints

Not all individuals who have cryoglobulinemia will have symptoms. However, when the disease worsens, symptoms such as these can appear:

The most severe symptoms are estimated to affect less than 1 in every 10 individuals diagnosed with cryoglobulinemia. 

Causes

There are three types of cryoglobulinemia. The type is connected to which kind of antibody is causing the disease. They include:

Diagnosis & Tests

The physician will first take a medical history and ask for a list of symptoms. Upon symptom presentation, they will try to determine if there are any underlying diseases that cause cryoglobulinemia such as infection with HCV or an underlying autoimmune disorder. A hepatitis C antibody test will be conducted to detect the presence of antibodies produced by the body in response to hepatitis C infection.

Since cryoglobulinemia is fundamentally a disease of the blood, health care professionals will request blood tests. These will test for the presence of cryoglobulins; however, the cryoglobulins test is known to return false negatives. Additional blood tests may also be ordered as follows:

Often a skin biopsy and/or kidney biopsy will be taken. The skin biopsy will look for nerve damage or skin inflammation and the kidney biopsy will look for a particular type of tissue inflammation called membranoproliferative glomerulonephritis or MPGN for short. Further tests include:

Combining test results is often required to obtain a firm diagnosis for cryoglobulinemia.

Treatment & Therapy

Once cryoglobulinemia is confirmed, treatment can begin. The type of treatment recommended will depend on two factors: the type of cryoglobulinemia (Type I, II, III) and the severity of symptoms. For mild cases, staying warm is often the best treatment. For cases linked to infection by the HCV virus, treating hepatitis C is the recommended best course of action.

For more severe cases of cryoglobulinemia where major organs or whole body systems are compromised, there are certain medications that can be prescribed, including these:

For Type I cases of cryoglobulinemia that are linked to cancer, treatment of the underlying cancer is necessary.

A procedure called plasmapheresis can also be performed. In this treatment, the plasma is removed from the body and the abnormal antibodies are removed. Then the plasma is reintroduced into the body or replaced with donated plasma, protein, or fluids.

There are some possible complications that can arise once an individual has been diagnosed with cryoglobulinemia, including heart disease, infected skin ulcers, gastrointestinal bleeding, acute liver failure or kidney failure and even death, but these complications are relatively rare.

So long as treatment is provided promptly and continued for as long as deemed medically necessary, the long-term outlook for individuals with cryoglobulinemia is considered to be very good. But in cases where the kidneys are compromised, the outlook is less promising. In these cases, kidney dialysis or a kidney transplant may be a necessary component of long-term treatment.

Prevention & Prophylaxis

The only potential way to prevent cryoglobulinemia at the current time is to avoid becoming infected with the hepatitis C virus or another ailment that may lead to the onset of cryoglobulinemia. Hepatitis C is commonly spread through the use of intravenous drugs which should be avoided. For mild cases, however, simply avoiding cold temperatures can often prevent symptoms of cryoglobulinemia from arising or returning.