Autoimmune Lymphoproliferative Syndrome (ALPS)

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About 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

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.

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

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

Treatment

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

Health Related Websites

Autoimmune Lymphoproliferative Syndrome information, Autoimmune Lymphoproliferative Syndrome articles, Autoimmune Lymphoproliferative Syndrome facts, Autoimmune Lymphoproliferative Syndrome research, Autoimmune Lymphoproliferative Syndrome news

The National Cancer Institute

The National Eye Institute

The National Heart, Lung, and Blood Institute

National Institute on Aging

National Institute of Allergy and Infectious Diseases

National Institute of Arthritis and Musculoskeletal and Skin Diseases

National Institute of Diabetes and Digestive and Kidney Diseases

National Institute on Drug Abuse

National Institute of Mental Health

National Institute of Neurological Disorders and Stroke

DISCLAIMER
The contents of this site are for informational purposes only.The content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health care provider for any questions you may have regarding a medical condition.

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