Autoimmune Lymphoproliferative Syndrome (ALPS)

Autoimmune Lymphoproliferative Syndrome (ALPS) is an inherited disorder of the immune system that affects both children and adults. In ALPS, unusually high numbers of white blood cells called lymphocytes accumulate in the lymph nodes, liver, and spleen, which can lead to enlargement of these organs. ALPS can cause numerous autoimmune problems such as anemia (low count of red blood cells), thrombocytopenia (low count of platelets), and neutropenia (low count of neutrophils, the most common type of white blood cell in humans).

Each of the three words in ALPS helps describe the main features of this condition. The word autoimmune identifies ALPS as a disease of the immune system. The word lymphoproliferative describes the unusually large numbers of white blood cells (called lymphocytes) stored in the lymph nodes and spleens of people with ALPS. The word syndrome refers to the many common symptoms shared by ALPS patients.

Common autoimmune problems in ALPS include: very low red blood cell counts that can make one weak; very low platelet counts that cause bruises and nose bleeds and may pose a risk for excessive bleeding; very low white blood cell counts which creates a risk for bacterial infection.

An enlarged spleen is common in patients with ALPS. Usually, it is not necessary to remove the spleen unless there are severe problems that are not responsive to treatment or if there is concern that the spleen may rupture due to massive enlargement.

Steroids such a prednisone are used to treat ALPS.

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