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Autoimmune Lymphoproliferative Syndrome (ALPS)

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What is ALPS?

ALPS is a rare disease that affects both children and adults. ALPS stands for Autoimmune Lymphoproliferative (lim-fo-pro-lif'-er-a-tive) Syndrome. Each of these three words helps describe the main features of this condition. The word autoimmune (self-immune) identifies ALPS as a disease of the immune system. The tools used to fight germs turn against our own cells and cause problems. The word lymphoproliferative describes the unusually large numbers of white blood cells (called lymphocytes (lim'-fo-sites)) stored in the lymph nodes and spleens of people with ALPS. The word syndrome refers to the many common symptoms shared by ALPS patients.

National Institutes of Health (NIH) Findings

  • ALPS is a disorder that typically develops in early childhood but can show up in adults.
  • ALPS is not cancer, and it is not contagious.
  • There is a wide spectrum of illness in ALPS. For some, it is very mild; for others, it is more severe.
  • Once a person has ALPS, he or she does not become sicker and sicker over time. In fact, the problems seem to lessen as children get to be teenagers and young adults.
  • Most people with ALPS have episodes of autoimmune problems (conditions in which the immune system attacks cells in the body). These can happen at any age, but they appear worse in childhood. Common autoimmune problems in ALPS include:
    • Very low red blood cell counts (hemolytic anemia) that can make one weak.
    • Very low platelet counts (immune-mediated thrombocytopenia, or ITP) that cause bruises and nose bleeds, and may pose a risk for hemorrhage (excessive bleeding). Little spots called petechiae (pet-eek'-ia) may also show up on the skin when platelets are low.
    • Very low white blood cell counts (autoimmune neutropenia), creating a risk for bacterial infection.
    • Less often, other autoimmune problems can occur in almost any organ - skin, liver, kidney, and nerves are examples.
  • Genetic mutations responsible for ALPS can be passed on from generation to generation or can occur spontaneously.
  • Immune systems in patients with ALPS are generally efficient in fighting infection.
  • An increase in certain types of white blood cells called alpha-beta double-negative T cells are elevated in ALPS patients.

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Last Updated October 05, 2008