- red blood cells
help transport oxygen to your tissues
platelets small tiny cells help to form blood
- white blood cells are the
soldiers in your immune system.
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
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
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
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
Cataracts of the eyes
- Mood swings
- The body starts to rely on the
steroids and the amount has to be slowly reduced.
- 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
Stem cell treatment can help
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.