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Autoimmune Lymphoproliferative Syndrome (ALPS)

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Ways to Manage ALPS

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

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 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

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

Other Treatments

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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Last Updated October 06, 2008