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Cold agglutinins are normally made by the immune system  in response to infection and cause red blood cells to clump together (agglutinate) at low temperatures.

In primary autoimmune hemolytic anemia, no underlying systemic disease explains the presence of autoantibodies, whereas secondary autoimmune hemolytic anemia results from a systemic disease. The autoantibody may be Immunoglobulin G (IgG), Immunoglobulin M (IgM), or, rarely, Immunoglobulin A (IgA); it may be warm reacting or cold reacting.

Secondary cold agglutinin disease is usually caused by infection and lymphoproliferative disorders. Monoclonal secondary disease is usually chronic, occurring in adults. Polyclonal secondary cold agglutinin disease, which occurs in children and young adults, is usually transient.

Prognosis of the disease may be associated with an excellent long-term prognosis when secondary to M pneumonia or viral infections. In children and young adults, the condition usually lasts 1-3 weeks; evidence of cold agglutinins disappears within 6 months.

Patients with the mildly to moderately severe primary (idiopathic) variety of cold agglutinin disease are expected to have a good long-term prognosis if excessive exposure to cold is avoided and with close medical surveillance for complications or progression to lymphoma.

Links to websites for further research

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Nuggets of Wisdom

Yes, there is such a thing as having an allergic type reaction to temperatures. This is defined as a physical urticaria. Other physical urticarias include Solar, Aquagenic, Pressure, Vibration and Exercise.

Most reactions are pseudo-allergic. By definition, an allergy involves inhaling or consuming an allergen. Physical urticarias have no known allergen. Despite the terminology and medical definitions, systemic reactions can be life threatening.

Cold has an arbitrary definition based on an individual feeling. For a person with a cold urticaria, cold can be defined as any temperature cooler than their own body temperature.

You do not have to be cold to have a reaction to the cold; contact with cold can trigger a reaction.

You can have an allergic type reaction to both cold and heat simultaneously.

Most reactions considered anaphylactic are really anaphylactoid by definition.

Moving to a warmer climate as a treatment for Cold Urticaria is a myth. Warmer climates present their own issues for those with Cold Urticaria.

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