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Cold agglutinin disease
Cold agglutinin disease is a rare type of autoimmune hemolytic anemia in which the body's immune system mistakenly attacks and destroys its own red blood cells
Prevalence
1-9 / 1 000 000
Age of Onset
ICD-10
D59.1
Inheritance
Autosomal dominant
Autosomal recessive
Mitochondrial/Multigenic
X-linked dominant
X-linked recessive
5 Facts you should know
FACT
Hemolysis in CAD is driven by C1 activation of the classical complement pathway, when cold agglutinins (IgM autoantibodies) recruit and activate C1, typically at body temperatures under 98.6 °F
FACT
Cold agglutinin disease can be primary or secondary, due to an underlying condition such as an infection, another autoimmune disease, or certain cancers
FACT
Primary cold agglutinin disease occurs after the fifth decade of life and peaks prevalence in a person's 70s and 80s
FACT
Many people with CAD also experience pain and bluish coloring of the hands and feet (acrocyanosis) or Raynaud's disease
FACT
Mycoplasma pneumoniae infection is the most common cause of secondary CAD
Interest over time
Common signs & symptoms
Tiredness
Dizziness
Headaches
Cold hands and feet
Pale skin
Dark urine
Jaundice
Chest pain
Current treatments
The treatment of cold agglutinin disease depends on many factors including the severity of the condition, the signs and symptoms present in each person, and the underlying cause. For example, in those affected by secondary cold agglutinin disease, it is important to diagnose and treat the underlying condition which may include certain types of cancer; bacterial, viral, or parasitic infections; and/or other autoimmune disease. People with few symptoms and/or mild anemia may not require any specific treatment. These cases are often managed by simply avoiding exposure to the cold.
Medical intervention
Rituximab
an antibody that selectively reduces specific types of immune cells