Frequently Asked Questions

What causes Cold Agglutinin Disease?

CAD, also known as primary chronic CAD, is a rare and serious autoimmune hemolytic condition with systemic acute and chronic consequences. Hemolysis in CAD is dependent on classical complement pathway activation.1,2

Unlike cold agglutinin syndrome (CAS), CAD is not caused by malignancy or acute infection.3

CAD comprises approximately 20% of all types of AIHA.4 Based on a population study, CAD affects up to 16 people per 1,000,000 with an average age of onset of 60 years, although onset of CAD has been seen in some patients as young as 30 years.1

AIHA=autoimmune hemolytic anemia; CAD=Cold Agglutinin Disease.
References: 1. Berentsen S, Ulvestad E, Langholm R, et al. Primary chronic cold agglutinin disease: a population based clinical study of 86 patients. Haematologica. 2006;91(4):460-466. 2. Berentsen S. Complement activation and inhibition in autoimmune hemolytic anemia: focus on cold agglutinin disease. Semin Hematol. 2018;55(3):141-149. 3. Berentsen S, Tjønnfjord GE. Diagnosis and treatment of cold agglutinin mediated autoimmune hemolytic anemia. Blood Rev. 2012;26(3):107-115. 4. Mullins M, Jiang X, Bylsma LC, et al. Cold agglutinin disease burden: a longitudinal analysis of anemia, medications, transfusions, and health care utilization. Blood Adv. 2017;1(13):839-848.