autoimmune lymphoproliferative syndrome (ALPS)
A rare 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).
How to manage ALPSThere is no cure for ALPS. However, most of its complications can be treated.
SplenectomyAn 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
Risks of splenectomy
SteroidsSteroids 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
Other treatmentsBlood 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.
ALPS and your family
Risk to childrenIndividuals with a Fas mutation have a 50/50 chance of passing the Fas mutation on to their children. This chance is the same for each child. Not all individuals with a Fas mutation develop ALPS.
Relatives who do not have ALPSSiblings or other relatives who don't have ALPS are still affected by the condition. Some worry that their brother or sister will die. Some think they will develop ALPS because they look or act like their brother or sister. Some children struggle with how much of their parents' time is spent with their sick brother or sister. It is important for families to talk openly about ALPS so that any misconceptions can be explained and feelings can be expressed. Family counseling is often helpful.
Many families say that ALPS has brought them closer together. Family members learn that they can rely on each other for support. Children learn from their parents' open and honest communication, and the experience teaches them how to solve problems.
Common questions and problems people with ALPS face
Why is your face so round?
People taking steroids may gain weight quickly. Some parents talk to teachers about the side effects of steroids in advance, so that classmates know what to expect. The swelling is temporary and goes away once the child stops taking the medicine.
Are you contagious?
People often worry that ALPS can spread to others, but ALPS is not infectious.
Stares from strangers
Parents of children with ALPS have to work extra hard to develop their children's self-confidence and sense of security. Children and adults with ALPS need to be reminded that they have many positive characteristics.
Spleens and sports
Some doctors recommend that people with enlarged spleens not play contact sports. When the spleen is large, it is fragile and there is a risk of rupture. NIH encourages use of a spleen guard, which is a piece of fiberglass that is molded to a person's stomach. It is easily wrapped around the stomach and held in place under a shirt. In general, the spleen guard is worn during any activity with a high risk for stomach injury, such as contact sports. Some people wear spleen guards while swimming, running track, or bicycling.
SymptomsNot 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:
Related category• HEALTH AND DISEASE
Source: National Institute of Allergy and Infectious Diseases
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