Understanding cold agglutinin disease


When the number of red blood cells in your body is too low, it can lead to anemia. It is estimated that more than 1.6 billion people around the world are suffering from anemia.

One type of anemia is called hemolytic anemia, or anemia caused by the destruction of red blood cells. There are different types of hemolytic anemias, including cold agglutinin disease (CAD).

Read on to learn more about this condition.

Coronary artery disease is a disease in which a disorder of the B-cell bone marrow causes autoimmune hemolytic anemia. It’s been about 15 percent autoimmune hemolytic anemias.

It usually occurs in people between the 40 and 80 years old and is more common in the elderly. The median age at which people are diagnosed is 65.

The prevalence of this condition is estimated at around 16 people per million. It develops in one person per million every year.

There are two types of CAD: primary and secondary. Primary CAD is when the triggering factor is unknown and there is no other condition that causes it. Secondary CAD is when associated with an underlying disorder. This happens until 70 percent people living with CAD.

The underlying disorders associated with coronary artery disease include:

Many people with coronary artery disease have symptoms of hemolytic anemia. Symptoms and their severity may vary depending on the severity of your anemia. These symptoms can include:

Some of these symptoms are caused by hemolysis, which is the destruction of red blood cells.

If you live with CAD, you may also have:

Your immune system usually produces antibodies that attach to invading cells and destroy them. When antibodies instead bind to red blood cells and think they are targets, coronary artery disease can develop. One of these antibodies is IgM, which causes many cases of coronary artery disease in humans.

When antibodies attack healthy tissue, they are called autoantibodies. When these autoantibodies are active in coronary artery disease, they can cause hemolysis if exposed to cold temperatures.

Once your red blood cells are labeled with a cold-induced antibody, they clump together and attach to proteins called supplements. These are also part of your immune system. When this happens, the red blood cells are then destroyed.

If the underlying cause of all of this is not known, it is considered a primary CAD.

When coronary artery disease is a secondary disorder, it is associated or caused by different disorders such as an infectious disease or a connective tissue disorder.

There are several factors that healthcare professionals can use to help diagnose coronary artery disease. These may include:

  • your detailed medical history
  • clinical evaluation
  • find characteristic symptoms
  • blood tests, especially those measuring your hemoglobin and hematocrit (the percentage of red blood cells present)

If a healthcare professional suspects that your hemolytic anemia is autoimmune, they Comb test. This test detects antibodies attached to your red blood cells or other biological components.

Once done, the medical expert will perform a thermal amplitude test, which examines blood samples at different temperatures. They will be able to see how your antibodies react to different temperatures. This determines how much cold agglutinin is present.

Treatment for coronary artery disease depends on the severity of the disease, the symptoms you have, and the underlying causes. If your symptoms are mild or the hemolysis seems to be slowing down, you may not need treatment.

If hemolysis increases, medication may be needed. Rituximab is the standard treatment for coronary artery disease. It can be combined with certain chemotherapy drugs or with prednisone.

Rituximab targets white blood cells which make the antibodies that destroy your red blood cells. Rituximab is also used to treat relapses.

If there is another condition causing CAD, that condition is treated.

If you have rapid hemolysis or severe anemia, you may need blood transfusions or a plasma exchange. But these don’t actually treat your anemia. They only relieve symptoms temporarily.

Other treatments include avoiding cold temperatures when possible, especially on the head, face and extremities. Preheating intravenous (IV) fluids is also recommended.

Risk factors can increase your chances of developing a disease. Just because you have one or more risk factors does not mean that you will permanently develop the disease. This means that you are more at risk.

Risk factors for coronary artery disease can include:

  • be a woman
  • have an associated condition previously listed
  • live in a colder climate

According to a 2020 report that categorized people into men and women, the CAD is almost twice as frequent in women than in men.

The outlook for people with CAD can vary widely. This can depend on factors such as the severity of the disease and your symptoms, and whether you have an underlying disease.

If an infection or unknown condition is causing coronary artery disease, your outlook is generally good to great. This is especially true if you are avoiding unnecessary exposure to cold temperatures.

But if HIV or certain types of cancer cause coronary artery disease, the outlook is generally less favorable. This is due to the characteristics of the underlying diseases.

Coronary artery disease is a rare type of anemia that can cause severe symptoms, leading to disease and impaired quality of life. Although it is not cancer, it can be caused by certain types of cancer, as well as a variety of other conditions.

Your treatment and outlook may vary depending on a variety of factors. If you have coronary artery disease, talk to your doctor about your treatment options and the steps you can take to minimize the symptoms.


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