Someone experiencing symptoms of cold agglutinin disease.

Symptoms and Causes of Cold Agglutinin Disease

According to the National Institute of Health, “Cold agglutinin disease is a rare type of autoimmune hemolytic anemia in which the body's immune system mistakenly attacks and destroys its own red blood cells.”

Those who are infected have IgM antibodies that attach to red blood cells and clump them together when their blood is exposed to low temperatures. This process of binding the blood cells into clumps is called agglutination. This results in the premature destruction of red blood cells which leads to anemia. Anemia occurs when there are not enough red blood cells to ensure oxygen is carried throughout the body.

Symptoms of Cold Agglutinin Disease

The symptoms of cold agglutinin disease can be either triggered or exacerbated by viral infections or cold temperatures. Therefore, the symptoms are often worse or more prevalent during the winter.

Most develop symptoms of hemolytic anemia. The severity and number of symptoms experienced often depends on the severity of the anemia itself. According to NIH, some of these symptoms include:

  • Tiredness (fatigue)
  • Dizziness
  • Headaches
  • Cold hands and feet
  • Pale skin
  • Dark urine
  • Jaundice
  • Chest pain
  • Pain in the back or legs
  • Vomiting or diarrhea
  • Heart problems such as an irregular heartbeat (arrhythmia), a heart murmur, an enlarged heart, or heart failure

Heart issues may occur due to the heart having to work harder to deliver oxygen to the body due to its loss of red blood cells. Enlargement of the spleen or discoloration of the skin may occur as well.

There is also a chance that one with cold agglutinin disease may experience discoloring in their hands and feet. When this happens one’s hands and feet may look like they have a blueish tint and they may feel pain there. This is because they are not receiving enough oxygen due to the decrease in red blood cells.

In those who have cold agglutinin disease associated due to a previous condition, it is called secondary cold agglutinin disease. The most common cause of secondary cold agglutinin disease is mycoplasma pneumoniae infection. If this is the cause of the CAD (cold agglutinin disease), then one may experience issues with their respiratory system as well. Other infections or cancers can result in the swelling of lymph nodes.

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Causes of Cold Agglutinin Disease

There are two types of classifications for CAD. The first is primary CAD. The cause of primary CAD is unknown. Secondary CAD can be associated with a wide array of causes.

As we discussed earlier, mycoplasma pneumoniae is one of the main causes of secondary CAD. However, there are other bacterial infections that can also cause CAD. Some of the other bacterial infections that can cause CAD include mycoplasma, legionnaires’ disease, syphilis, listeriosis, or E. Coli.

Viral infections like hepatitis C, the flu, Epstein-Barr virus, cytomegalovirus, mumps, rubella, varicella, adenovirus and HIV can all have a chance of causing secondary CAD. Malaria, trypanosomiasis and systemic lupus erythematosus also have a chance of causing secondary CAD.

Cancers such as lymphoma, chronic lymphocytic leukemia, Waldenstrom macroglobulinemia, multiple myeloma and Kaposi sarcoma are ones that can cause secondary CAD.

Treatment for Cold Agglutinin Disease

The treatment of CAD depends on a large variety of factors. The symptoms one’s experiencing, the severity of the symptoms and the underlying cause all determine what course of treatment may be best.

If one has secondary CAD, then figuring out the root cause of their CAD is one of the first steps. After identifying what the underlying cause is for secondary CAD, then it is important to treat that (whether it be a bacterial infection, viral infection or cancer).

Those with only a few symptoms or moderate anemia may not need any specific care. In this case, these people may just be told to avoid the cold. However, for those with more severe symptoms of hemolysis, it is important that they receive care from a medical professional.

In severe cases, your doctor may consider injecting you with the antibody, Rituximab. These injections help reduce specific types of cells within the immune system that may be exacerbating your symptoms. It has been found to be effective in around 60% of cases.

Living with CAD

CAD can be a difficult ailment to deal with, especially if you live in a place with colder seasons. While there are measures one can take to try and reduce the risk of anemia that can result from CAD, sometimes treatment is required.

If you feel you may be experiencing any of the symptoms of CAD, it may be best to take a trip to your doctor. They will be able to determine what changes (if any) that you need to make to improve your condition, as well as whether your condition may require any medications or treatment procedures. They also may be able to connect you with support groups that help others deal with some of the symptoms you may be dealing with as well.