Cold agglutinin disease, or CAD, is a rare autoimmune disorder in which self-targeting antibodies attack and destroy red blood cells at low temperatures.
Types of CAD
CAD can be divided into two types — primary and secondary CAD — depending on the underlying cause. In secondary CAD, the disease develops in association with another health condition, such as an infection or cancer. With primary CAD, the specific cause is unknown and other illnesses are not known to exist.
Causes of CAD
CAD is caused by a specific type of self-reactive antibodies called cold agglutinins. At cold temperatures — generally between 32 to 50 F (0 to 10 C) — cold agglutinins bind to red blood cells, causing them to clump together, or agglutinate, and ultimately die.
Symptoms of CAD
Red blood cells are responsible for carrying oxygen through the blood to all body tissues. In CAD and other types of anemia, tissues often do not receive enough oxygen, which can lead to fatigue and pain.
A common symptom of CAD is Raynaud’s syndrome, a condition in which blood vessels — usually in the fingers and toes — constrict when exposed to cold temperatures. Affected areas may turn pale, red, or blue, and patients may experience a numb or tingling sensation.
Red blood cell destruction can also cause the skin to become unusually pale or yellowish (jaundice). Some CAD patients may also develop acrocyanosis, or bluish skin discoloration.
Diagnosis of CAD
CAD usually is first suspected when a person notices certain symptoms when exposed to cold, or when a physical exam reveals potential signs of anemia. A variety of laboratory tests can be done on a patient’s blood to confirm the presence of anemia and detect cold agglutinins, confirming a diagnosis.
In mild cases, CAD may not require medical treatment, with symptoms sufficiently controlled by avoiding exposure to cold temperatures and taking additional precautions under specific circumstances, including during hospitalization.
Taking supplements of folic acid, a man-made version of a vitamin the body uses to make red blood cells, may also help to alleviate symptoms of anemia. Treatment with a hormone called erythropoietin may help to spur the production of new red blood cells.
In more severe cases, medications like rituximab, which works by destroying antibody-producing immune B-cells, may be needed. Rituximab may be used on its own to treat CAD, or in combination with other therapies like fludarabine.
Last updated: Oct. 15, 2021
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