Autoimmune Lymphoproliferative Syndrome (ALPS) is an inherited disorder of the immune system that affects both children and adults. In ALPS, unusually high numbers of white blood cells called 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 anemia (low count of red blood cells), thrombocytopenia (low count of platelets), and neutropenia (low count of neutrophils, the most common type of white blood cell in humans).
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
* Thombocytopenia (low platelet count), which can cause bruising, nose bleeds, and may pose a risk for hemorrhage (excessive bleeding); little red spots called petechiae (pet-eek’-ia) may also show up on the skin when platelets are low
* Anemia (low red blood cell count), which can cause increased fatigue or pallor
* Neutropenia (low neutrophil count), which can create a risk for bacterial infections
There is no cure for ALPS. However, most of its complications can be treated.
An enlarged spleen is common in patients with ALPS. Usually, it is not necessary to remove the spleen unless there are severe problems like anemia 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.
Benefits of Splenectomy
* Easier to regulate and control blood counts
* Less discomfort
* No risk of spleen rupture
Risks of Splenectomy
* Increased likelihood of certain bacterial infections, which can be life threatening; patients must get vaccinated to avoid infections, and some may need to take antibiotics for many years
* Possible recurrence of anemia or thrombocytopenia
Steroids are the first line of treatment for anemia 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.
When prednisone is not enough to treat these episodes, other drugs such as mycophenolate mofetil, rituximab, IVIG, and vincristine may also be prescribed. Steroids have been very effective in treating these problems. However, steroids can have adverse side effects, so they should not be used for extended periods of time.
Possible Long-Term Side Effects of Steroids
* Thinning of bones
* Poor wound healing
* Difficulty fighting infection
* Cataracts of the eyes
* Mood swings
* Weight gain
* Elevated blood sugar
Blood transfusions are useful to replace red blood cells when anemia 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.
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