About Autoimmune Lymphoproliferative Syndrome (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.

Statistics About Autoimmune Lymphoproliferative Syndrome (ALPS)

At this time we are still learning about ALPS. Based on our experience, we believe the following to be true:

• ALPS is a disorder that develops in early childhood.
• ALPS is not cancer; it is not contagious; it is not AIDS.
• 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 improve as children get to be teenagers and young adults.
• Most people with ALPS have episodes of autoimmune problems. These can happen at any age, but they appear worse in childhood.

Types of Autoimmune Problems

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.  

Autoimmune Lymphoproliferative Syndrome (ALPS)-Immunology

To understand what happens in ALPS, we would like to review some basic information about the immune system.

Causes of Autoimmune Lymphoproliferative Syndrome (ALPS).  To better understand how ALPS works, imagine that you have a respiratory infection, perhaps the flu.

Management of Enlarged Spleens in Autoimmune Lymphoproliferative Syndrome (ALPS)

Virtually all people with ALPS have an oversized spleen. Usually, it is not necessary to remove the spleen unless there are severe problems like anemia. However, removing a spleen carries both risks and benefits, which doctors and patients must carefully consider before deciding what to do.

Management of Autoimmune Problems in Autoimmune Lymphoproliferative Syndrome (ALPS)

Steroids are the first line of treatment for autoimmune episodes, like hemolytic anemia and ITP. One common steroid is prednisone. 

• Blood Transfusions are useful to replace red blood cells when anemia is severe.
• Vaccines are important to help prevent infections. The fewer infections you have, the less often you will need to "call in the troops." In addition to all the childhood vaccinations, it is important to get a yearly flu shot and boosters as needed. People with allergies to eggs should discuss this with their doctor prior to receiving a flu shot.
• Gene Therapy is unfortunately not likely to work for ALPS.

References for Autoimmune Lymphoproliferative Syndrome (ALPS) Article

• National Institute of Allergy and Infectious Diseases