Cold Agglutinin Disease -What is Cold Agglutinin Disease? A form of autoimmune hemolytic
anaemia, specifically one in which antibodies only bind red blood cells at low body
temperatures.
Women are affected more commonly than men.
There are two forms of cold agglutinin disease:
Primary - idiopathic, a disease for which no cause is known.
Secondary - 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 it is often secondary to an infection, such as Mycoplasma
pneumonia, mononucleosis, and HIV.
The condition is more likely to attack cooler parts of the body such as the fingers,
nose, and ears.
The immune system produces antibodies that attack the red blood cells when the body
is cold, causing the symptoms of the disease to develop. Normally, red blood cells
have a life span of approximately 120 days before the spleen removes them from the
body.
When individuals have cold agglutinin disease, the red blood cells are destroyed
prematurely and bone marrow production of new cells can not compensate for their
loss.
The severity of the anaemia that develops is determined by the length of time that
the red blood cells survive and by the ability of the bone marrow to continue red
blood cell production.
Uneven bluish or reddish discolouration of the skin of the fingers, toes, ankles
and wrists.
Some with Cold Agglutinin Disease may also be affected by two conditions - Raynaud’s
phenomenon and acrocyanosis.
Raynaud’s phenomenon is triggered when cold constricts small arteries usually in
the fingers and toes but can also affect other parts of the body such as the nose,
ears, and face.
Acrocyanosis - a painless disorder caused by constriction or spasms of small blood
vessels due to cold. Those with the disease tend to be uncomfortable and may experience
swollen, sweaty, cold, bluish coloured fingers, hands and feet.
