What is Autoimmune Lymphoproliferative Syndrome?

Written by Medicover Team and Medically Reviewed by Dr Lavanya Garapati , Hematologists

---

Autoimmune Lymphoproliferative Syndrome, or ALPS, is a rare genetic disorder that affects the way the immune system controls the growth and death of certain white blood cells called lymphocytes. In people with ALPS, the body has trouble turning off or removing these cells when they're no longer needed.

As a result, these cells collect in the body and may begin to attack healthy tissues, putting stress on the immune system. One of the first signs families may notice is swollen lymph nodes or an enlarged spleen. ALPS often starts in childhood, but the symptoms can last into adult life. Even though it is rare, finding it early helps doctors treat it better and improve long-term health.

What are the Common Signs and Symptoms of Autoimmune Lymphoproliferative Syndrome?

Autoimmune Lymphoproliferative Syndrome (ALPS) typically presents in early childhood and is marked by chronic enlargement of lymph nodes, spleen, or liver. The symptoms of ALPS can vary but often include:

These symptoms might appear gradually, and some may be mistaken for more common childhood illnesses or blood disorders.

---

What are the Causes of ALPS?

The condition is usually caused by a mutation in genes that regulate a natural process called apoptosis, the programmed death of immune cells. When this process doesn't work, immune cells survive longer than they should and can begin to misbehave.

Most cases are linked to mutations in the FAS gene, although other genes may be involved. ALPS can be inherited from a parent, or the mutation can occur spontaneously in a child with no family history.

---

How is ALPS Diagnosed?

Diagnosing ALPS can be challenging, especially since the symptoms overlap with other immune conditions. Doctors typically begin with a full medical history and physical exam, followed by specific tests:

Early diagnosis helps reduce the risk of complications and guides appropriate treatment.

---

What are the Treatment and Long-Term Management of ALPS?

There is no permanent cure for ALPS, but many people respond well to medical treatment aimed at controlling the overactive immune response.

Corticosteroids are often used to manage autoimmune symptoms. Immunosuppressive medications, such as sirolimus or mycophenolate, may be prescribed for long-term control.

In some cases, splenectomy (removal of the spleen) might be considered. Most individuals with ALPS can lead normal lives with ongoing care and monitoring.

---

When to See a Doctor

You should see a doctor if your child has persistently swollen lymph nodes, an enlarged spleen or liver, or unexplained fatigue, bruising, or frequent infections. Early medical evaluation is essential, especially if there's a family history of immune system disorders.

You should consult a specialist if a child or adult has:

---

Our Experience in Treating ALPS

At Medicover Hospitals, our expert doctors in immunity, blood disorders, and genetics work closely to find the right diagnosis and create a care plan that fits each patient. With advanced tests and kind support, we help people manage ALPS and feel better over time. Early treatment, the right care, and a caring team can make life much easier for those living with this condition.

---