Cold Agglutinin Disease in Children

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by Mary Chapman |

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pediatric CAD

While the risk for cold agglutinin disease (CAD) increases after age 55, children occasionally develop the disorder. In these cases, CAD is usually the result of infection, lasts a relatively short time, and generally requires supportive care only.

What is CAD?

CAD is a type of autoimmune hemolytic anemia, in which the immune system produces antibodies called cold agglutinins. These antibodies mistakenly attack red blood cells at low temperatures and cause them to burst, a process called hemolysis.

The disease may manifest as a primary disease, in which the underlying cause is unclear, or as a secondary disease caused by underlying medical issues such as infections, certain types of cancer, or other autoimmune diseases.

Can children have CAD?

CAD is a relatively rare disorder in children that doctors most often diagnose after a recent infectious illness.

Researchers estimate the number of patients, from 11 to 20 years of age, with all types of autoimmune hemolytic anemia to be approximately 0.2 per 100,000.

How do doctors treat CAD in children?

Secondary CAD caused by a non-viral infection is usually transient, although antibiotics may be beneficial. These patients rarely need immunosuppressive treatment or plasmapheresis — a process that filters the blood and removes harmful antibodies — unless acute anemia is present.

What happened in some case studies?

A study reported the case of a girl, age 9, who developed cold agglutinin-induced acrocyanosis — a bluish skin discoloration due to the blockage of small blood vessels — after a respiratory infection. Doctors prescribed the girl an antibiotic and a medication to improve blood flow. They advised her to avoid cold and to keep her extremities warm. After six months, she showed only mild symptoms.

In another study, doctors diagnosed a girl, 8, with CAD apparently brought on by an acute Epstein-Barr virus infection. She recovered uneventfully after a three-week course of corticosteroid treatment.

Cases of cold antibody autoimmune hemolytic anemia secondary to infection are mostly mild and self-limiting. However, they can occasionally be severe. One study reported the case of a boy, 3, who had recently had an infection and was hospitalized with a fever and life-threatening acute hemolysis. Unresponsive to high-dose steroids and intravenous immunoglobulin, he recovered following plasmapheresis.


Last updated: Aug. 27, 2020


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