Etiology
There are two forms of cold agglutinin disease: 1-primary and 2-secondary.
- The primary form is by definition idiopathic, a disease for which no cause is known.
- Secondary cold agglutinin disease is a result of an underlying condition.
- In adults, this is typically due to a lymphoproliferative disease such as lymphoma and chronic lymphoid leukemia, or infection.
- In children, cold agglutinin disease is often secondary to an infection, such as Mycoplasma pneumonia, mononucleosis, and HIV.
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