Cold Agglutinin Disease: Symptoms & Signs

  • Medical Author:
    Melissa Conrad Stöppler, MD

    Melissa Conrad Stöppler, MD, is a U.S. board-certified Anatomic Pathologist with subspecialty training in the fields of Experimental and Molecular Pathology. Dr. Stöppler's educational background includes a BA with Highest Distinction from the University of Virginia and an MD from the University of North Carolina. She completed residency training in Anatomic Pathology at Georgetown University followed by subspecialty fellowship training in molecular diagnostics and experimental pathology.

Medically Reviewed on 2/28/2019

Cold agglutinin disease is a rare form of autoimmune hemolytic anemia. In this condition, upon exposure to cold temperatures, the body's immune system mistakenly attacks and destroy its own red blood cells (erythrocytes). Cold agglutinin disease may occur on its own (primary) due to unknown causes, or it may be secondary to other diseases.

Signs and symptoms of cold agglutinin disease include fatigue, cold feet and hands, dizziness, dark urine, pale skin, and headaches. Other possible associated symptoms and signs can include yellowed skin and eyes (jaundice), chest pain, leg pain, back pain, vomiting, and diarrhea. In general, people with any type of anemia, including cold agglutinin disease, may have other symptoms like a rapid heart rate, heart murmur, palpitations, or shortness of breath.

Causes of cold agglutinin disease

The cause of cold agglutinin disease is unknown.


Kasper, D.L., et al., eds. Harrison's Principles of Internal Medicine, 19th Ed. United States: McGraw-Hill Education, 2015.

Medically Reviewed by a Doctor on 2/28/2019

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