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• red blood cells help transport oxygen to your tissues
• platelets small tiny cells help to form blood clots
• white blood cells are the soldiers in your immune system.

One major class of white blood cells is called lymphocytes. There are many kinds of lymphocytes to combat the variety of germs in the world. B cells and T cells are two major kinds of lymphocytes.

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. The Inflammation continues.

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.

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 (see WHAT HAPPENS IN ALPS).
• 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.

You can avoid splenectomy by using IVIG

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.

IVIG can help all issues with ALPs specially the autoimmune responce.

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 reduced.

TREATMENTS

• IVIG INFUSION, 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.
• Stem cell treatment can help

DOES ALPS RUN IN FAMILIES?

Children can inherit ALPS from one of their parents.

A BRIEF INTRODUCTION TO GENETICS

Genes, which are made of DNA, are located on chromosomes in the cells of our body.. By chance, every person has approximately 10-12 genes that don't work as well as they should or don't work at all. These genes are mutated. That is, the DNA is slightly different in mutated genes. Not all changes, or mutations, are harmful. Sometimes these changes cause no problems at all, but sometimes they cause human disease. Sometimes it takes alterations in several genes to cause problems. Other times the genetic mutation interacts with the environment to cause health problems.