Autoimmune lymphoproliferative syndrome


Autoimmune lymphoproliferative syndrome (ALPS) is a rare autoimmune condition that usually develops in early childhood (before five years of age) and that affects both children and adults. In patients who develop the disease later in life, the symptoms are usually not as severe. ALPS is an inherited condition that affects both sexes.
Normally, the immune system attacks foreign invaders, such as bacteria. An autoimmune condition occurs when the immune system malfunctions and begins to attack normal components of the body. Antibodies are a normal component of the immune system that normally attack foreign invaders to help destroy them and protect the body. In patients with ALPS, there are increased numbers of lymphocytes, a type of white blood cell that makes antibodies. Some of the antibodies produced by the extra lymphocytes attack normal components of the body (because the antibodies are able to recognize proteins on these components), causing them to malfunction.
In patients with ALPS, antibodies commonly attack red blood cells, which transport oxygen throughout the body, and platelets, which help with clotting. However, the antibodies may attack cells in almost any location throughout the body, such as the skin, kidney, liver, or nerves. Patients with ALPS may have a wide spectrum of symptoms, depending on which components of the body have been attacked by the immune system. Some common symptoms may include weakness (due to reduced numbers of red blood cells), bruises or nosebleeds (due to reduced numbers of platelets), or increased risk of bacterial infection (due to decreased numbers of white blood cells).
ALPS generally does not lead to death and most individuals with ALPS are able to live normal lives. Currently, there is no known cure for ALPS. However, treatments exist that may help patients manage some of the symptoms of ALPS, including steroids that suppress the immune system.

Related Terms

ALPS, antibody, autoimmune condition, Canale-Smith Syndrome, fas, immune system, lymph node, lymphocyte, platelet, red blood cell, spleen, steroids, white blood cell.