Causes of Autoimmune Lymphoproliferative Syndrome


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Cause details for Autoimmune Lymphoproliferative Syndrome: To better understand how ALPS works, imagine that you have a respiratory infection, perhaps the flu. The cells in the nose and throat send out a message to the immune system to start making more lymphocytes to fight the flu. New troops of lymphocytes come to the nose and throat to seek out and destroy the cells infected with the flu virus. Once the virus is conquered, the lymphocytes get a message that their job is done and they are no longer needed. At this point, it is normal for most of the fighter cells to disintegrate through a process called apoptosis (a-pop-to'-sis).

The immune systems of people with ALPS are efficient in fighting germs. The problem in ALPS happens after an infection is gone. In ALPS, apoptosis does not work as well as it should. In other words, the troops (lymphocytes) donŐt hear the message that the war is over. As a result, excess T and B cells gather in the lymph glands, liver and spleen. We can detect the extra cells in people with ALPS by looking for high numbers of double-negative T cells. In general, these extra T cells donŐt cause a problem.

Sometimes in ALPS, the B cells make a mistake. Instead of making antibodies to be custom-designed against germs, the B cells make antibodies against platelets, red blood cells, or other cells. This causes autoimmune problems. The antibodies become stuck to the platelets and red blood cells, which then get stuck in the spleen. The spleen has to work extra hard to filter out the sticky cells. This is another reason why the spleen gets so big.1

Related information for causes of Autoimmune Lymphoproliferative Syndrome: Further relevant information on causes of Autoimmune Lymphoproliferative Syndrome may be found in the risk factors for Autoimmune Lymphoproliferative Syndrome and underlying causes of Autoimmune Lymphoproliferative Syndrome.

Footnotes:
1. excerpt from Autoimmune Lymphoproliferative Syndrome (ALPS): NIAID

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