Autoimmune Lymphoproliferative Syndrome (ALPS) : Management

by National Institute of Health

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There are many kinds of T cells. Some of these direct our immune response to infections, and others kill infected cells. Each T cell is named for the markers found on its surface. There are over 100 different markers. For example, some T cells are called CD4 cells because they have the type 4 chemical marker coating their surfaces. Other T cells are named CD8 cells because they are coated by the type 8 marker. Almost all T cells of healthy people have either the type 4 or the type 8 marker. Those with neither type 4 nor type 8 markers are called double-negative T cells. People with ALPS often have increased numbers of double-negative T cells.

People with ALPS often have extra B cells, too. The B cells produce immunoglobulins (Ig's, also called antibodies). The antibodies are custom-fit to stick to specific germs. There are thousands of different antibodies in the body. Each is molded for a specific germ. Because ALPS patients have more B cells than normal, they produce more antibodies, including ones that cause autoimmune problems.

What Happens In ALPS

To better understand how ALPS works, imagine that you have a respiratory infection, perhaps the flu. The cells in the nose and throat send out a message to the immune system to start making more lymphocytes to fight the flu. New troops of lymphocytes come to the nose and throat to seek out and destroy the cells infected with the flu virus. Once the virus is conquered, the lymphocytes get a message that their job is done and they are no longer needed. At this point, it is normal for most of the fighter cells to disintegrate through a process called apoptosis (a-pop-to'-sis).

The immune systems of people with ALPS are efficient in fighting germs. The problem in ALPS happens after an infection is gone. In ALPS, apoptosis does not work as well as it should. In other words, the troops (lymphocytes) don't hear the message that the war is over. As a result, excess T and B cells gather in the lymph glands, liver and spleen. We can detect the extra cells in people with ALPS by looking for high numbers of double-negative T cells. In general, these extra T cells don't cause a problem.

Sometimes in ALPS, the B cells make a mistake. Instead of making antibodies to be custom-designed against germs, the B cells make antibodies against platelets, red blood cells, or other cells. This causes autoimmune problems. The antibodies become stuck to the platelets and red blood cells, which then get stuck in the spleen. The spleen has to work extra hard to filter out the sticky cells. This is another reason why the spleen gets so big.

Management of ALPS

There is no cure for ALPS. However, we can treat and prevent most of its complications. Management of ALPS involves:

Diagnosis. You probably know from experience that this may take months or years until you find a doctor who recognizes the features of ALPS.

Counseling and education. The more you know about ALPS and how to recognize its symptoms and signs, the better you will be able to manage it.

Knowing what's treatable. Unfortunately, we have not found ways to permanently make the swelling of lymph nodes go down or to fix the problem with apoptosis.

Therapies. Complications of ALPS, including the many different autoimmune problems, can be treated successfully. ALPS can be managed through close communication with doctors as symptoms and signs arise.

Ways to Manage Enlarged Spleens in 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.

Benefits of Splenectomy

• It will be easier to regulate and control blood counts.
• You will feel less discomfort.
• There is no longer a risk of spleen rupture, a very serious problem, should it occur.

Risks of Splenectomy

You will be missing an organ which helps protect against infection. Your chances of getting certain bacterial infections increase. You must get some vaccines to avoid these infections.

After your spleen is removed, you may need to take antibiotics for many years to help prevent specific bacterial infections.

Ways to Manage Autoimmune Problems in ALPS

Steroids are the first line of treatment for autoimmune episodes, like hemolytic anemia and ITP. One common steroid is prednisone. It is often given for a short time, but sometimes it is needed for longer periods. When prednisone is not enough to treat the episode, other drugs, such as Imuran and cyclosporin, may also be prescribed. Steroids have saved lives and have dramatically reduced the complications in some people with ALPS. However, like all treatments, steroids have some disadvantages, so they should not be used too much or for too long.

Possible Long-Term Side Effects of Steroids

• Thinning of bones
• Poor wound healing
• Difficulty in fighting infection
• Diabetes
• Cataracts of the eyes
• Mood swings
• Weight gain
• The body starts to rely on the steroids and the amount has to be slowly reduce

About the Author

NIH is the nation's medical research agency - making important medical discoveries that improve health and save lives. The National Institutes of Health (NIH), a part of the U.S. Department of Health and Human Services, is the primary Federal agency for conducting and supporting medical research.