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Autoimmune Lymphoproliferative Syndrome (ALPS)

  • Posted on- Oct 23, 2015
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Autoimmune Lymphoproliferative Syndrome is a rare inherited disorder of the immune system that affects both children and adults. Each of these three words helps describe the main features of this condition. The word autoimmune (self-immune) identifies ALPS as a disease of the immune system. The tools used to fight germs turn against our own cells and cause problems. The word lymphoproliferative describes the unusually large numbers of white blood cells (called lymphocytes stored in the lymph nodes and spleens of people with ALPS. The word syndrome refers to the many common symptoms shared by ALPS patients.

In ALPS, unusually high numbers of lymphocytes accumulate in the lymph nodes, liver, and spleen, which can lead to enlargement of these organs. ALPS can cause numerous autoimmune problems such as anaemia (low count of red blood cells), thrombocytopenia (low count of platelets), and neutropenia (low count of Neutrophil, the most common type of white blood cell in humans).

Technical facts about Autoimmune Lymphoproliferative Syndrome

  • ALPS is a disorder that typically develops in early childhood but can show up in adults.
  • ALPS is not cancer, and it is not contagious.
  • 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 lessen as children get to be teenagers and young adults.
  • Most people with ALPS have episodes of autoimmune problems (conditions in which the immune system attacks cells in the body). These can happen at any age, but they appear worse in childhood.
  • Genetic mutations responsible for ALPS can be passed on from generation to generation or can occur spontaneously.
  • Immune systems in patients with ALPS are generally efficient in fighting infection.
  • An increase in certain types of white blood cells called alpha-beta double-negative T cells are elevated in ALPS patients.

Symptoms of Autoimmune Lymphoproliferative Syndrome

Not all people with ALPS will have all of its symptoms some people have only a few. Signs of ALPS that are seen most often include the following:
  • Enlarged spleen
  • Enlarged lymph nodes, especially in the neck and underarms
  • Enlarged liver
  • Skin rashes
  • Thrombocytopenia (low platelet count), which can cause bruising, nosebleeds, and may pose a risk for haemorrhage (excessive bleeding) little red spots called petechiae may also show up on the skin when platelets are low
  • Anaemia (low red blood cell count), which can cause increased fatigue or pallor
  • Neutropenia (low Neutrophil count), which can create a risk for bacterial infections

How to manage Autoimmune Lymphoproliferative Syndrome

There is no cure for ALPS. However, most of its complications can be treated.

Steroids
Steroids are the first line of treatment for anaemia and thrombocytopenia caused by autoimmune processes. One common steroid is prednisone. It is often given for a short time, but sometimes it is needed for longer periods.

Steroids have been very effective in treating these problems. However, steroids can have adverse side effects such as thinning of bones, weight gain, mood swings, high blood sugar, so they should not be used for extended periods of time.

Splenectomy
An enlarged spleen is common in patients with ALPS. Usually, it is not necessary to remove the spleen unless there are severe problems like anaemia and thrombocytopenia that are not responsive to treatment or if there is concern that the spleen may rupture due to massive enlargement. Removing a spleen carries both risks and benefits, which doctors and patients must carefully consider before deciding what to do.

Other Treatments
When prednisone is not enough to treat these episodes, other drugs such as mycophenolate mofetil, rituximab, immunoglobulin, and vincristine may also be prescribed.

Blood transfusions are useful to replace red blood cells when anaemia is severe. Vaccines are important to help prevent infections. In addition to all childhood vaccinations, it is important to get a yearly flu shot and boosters as needed. People with allergies to eggs should discuss the risks with their doctor prior to receiving a flu shot.