Jaundice in Cold Agglutinin Disease

Cold agglutinin disease (CAD) is a rare autoimmune disorder that can cause multiple symptoms, including jaundice.

In CAD, the immune system generates antibodies against red blood cells, causing them to burst in cold temperatures. Many people with CAD avoid cold temperatures, even before receiving a diagnosis, because cold makes their symptoms worse.

What is jaundice?

Jaundice is yellowing of the skin, eyes, and mucous membranes because of high levels of an orange-yellow protein called bilirubin. Bilirubin is a waste product created by the breakdown of red blood cells. Normally, red blood cells have a lifespan of about four months, after which they are recycled.  During the process of breaking down the worn red blood cells, bilirubin is produced as a waste product. Bilirubin passes through the liver and is excreted.

Jaundice and CAD

In CAD, the rapid breakdown of red blood cells means that far more bilirubin than normal is produced during cold exposure. The liver cannot process the bilirubin as fast as it is being released, and this causes the skin and eyes to acquire a yellow-orange tint.

How is jaundice diagnosed?

Usually, jaundice is a very visible condition — patients’ skin and the whites of their eyes become yellow. However, to confirm the cause of jaundice, individuals may need to undergo a blood test to check their bilirubin levels. The levels of other proteins produced by the liver, such as albumin, are usually checked at the same time. A blood cell count also may be performed to assess whether red blood cells are being attacked.

Jaundice also can be caused if the liver is not processing waste products normally. That causes waste like bilirubin to build up in the blood. To ensure that the liver is functioning normally, physicians will often perform liver function tests, even in patients who have already been diagnosed with CAD.

How is jaundice treated?

In adults, jaundice is not usually treated directly, unless there are underlying problems that need to be addressed, such as liver disease.

Children and infants with jaundice may be treated with phototherapy, in which special blue light lamps are used to break down the bilirubin. Extreme cases may be treated with intravenous immunoglobulin (IVIG) — in which the needed proteins are injected into the vein — or an exchange transfusion. In that treatment, small amounts of blood are taken over time and replaced with donor blood.


Last updated: August 7, 2019


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