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What Is Cold Agglutinin Disease?

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  • Written By: Andy Josiah
  • Edited By: Kaci Lane Hindman
  • Last Modified Date: 26 November 2016
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Cold agglutinin disease is a type of autoimmune disease that is characterized by antibodies agglutinating, or sticking to, red blood cells (RBCs). It is a variation of autoimmune hemolytic anemia, in which agglutination leads to the red blood cells dying — an occurrence known as hemolysis. Cold agglutinin disease is set apart from autoimmune hemolytic anemia, however, in that the antibodies only stick to red blood cells at low temperatures, at the approximate range of 39.2 to 98.6°F (4 to 37°C). Thus, such antibodies are referred to as cold agglutinins.

In 1903, Austrian-born Jewish American physician and chemist Karl Landsteiner made the first description of the existence of cold agglutinins. It was in 1957, however, that a trio of medical researchers with the surnames of Dacie, Crookston and Christenson made the first report of cold agglutinin hemolysis, thus marking the first description of cold agglutinin disease. Eventually, the antibody most commonly associated with the medical condition became immunoglobulin (Ig), usually the IgA, IgG or IgM variant.

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Two forms of cold agglutinin disease exist: primary disease and secondary disease. The primary type is described as idiopathic and chronic, meaning that its cause is unknown and that it is long lasting. Primary disease occurs from monoclonal antibodies, or antibodies that originate from the same cell. Secondary disease is usually triggered from another condition, usually another autoimmune disease, infection or a tumor. Also, it is typically generated by both monoclonal and polyclonal antibodies, the latter originating from different cells.

Cold agglutinin disease is most common in the elderly, particularly people in their 60s and 80s. The secondary polyclonal form of the disease in particular often affects younger people. In terms of gender, females are more commonly affected than males.

The most common symptom of cold agglutinin disease is anemia, which is the reduction of the number of RBCs or the amount of hemoglobin in the blood. Other common signs include painful and discolored toes and fingers; chronic fatigue; hemoglobinuria, or the release of high concentrations of hemoglobin from urine; and respiratory problems. People with the primary form of cold agglutinin disease might have the symptoms worsen during colder weather due to the low temperatures under which the cold agglutinins work optimally.

The best treatment for cold agglutinin disease is prevention. This means that staying away from cold weather or dressing warmly are the most effective measures people can take. In more extreme cases, however, physicians rely on the monoclonal antibody Rituximab, steroid hormones such as glucocorticoids, and immunosuppressive and immunomodulating drugs. Such treatments are used sparingly though, due to questionable effectiveness or their potentially harmful side effects.

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Drentel
Post 3

I think people with blood disorders that involve the red blood cells generally fair better in warmer climates. I have a friend who has poor circulation of the blood. He has few complaints when the weather is warm, but when we get cold weather he suffers a bit.

Animandel
Post 2

@Sporkasia - Moving to warmer climates is more than simply a way for older people to be more comfortable. This type of move can actually improve the quality of their lives and their health. Some people with the cold agglutinin disease will actually turn a very noticeable purple color when they are outside in cold weather.

One of my mother's friends would go outside during the winter months and when she came back inside her arms, fingers and toes would all be purple. Her ears and nose would be even more discolored. She moved to Florida, and while she still has some issues with the condition, the symptoms are not as bad as they were when she was going through the cold winters.

Sporkasia
Post 1

I work with a women who told me last week that her mother has this cold agglutinin disease. This woman's mother had been experiencing really bad back and leg pain for some time, and doctors had been trying to find the root of her problems.

Unfortunately, as people age they sometimes think that the pain is natural, and this is what this woman's mother was beginning to believe. It wasn't until she started having other symptoms that the doctors made this diagnosis.

Since the women lives in a place with very harsh winters, her daughter has suggested that a move to a warmer climate might be a good idea. This article seems to back up this theory.

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