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Autoimmune lymphoproliferative syndrome (ALPS)
Autoimmune lymphoproliferative syndrome (ALPS) is characterized by dysregulation of the immune system due to an inability to regulate lymphocyte homeostasis through the process of lymphocyte apoptosis
Prevalence
N/A
US Estimated
N/A
Europe Estimated
Age of Onset
ICD-10
D47.9
Inheritance
Autosomal dominant
Autosomal recessive
Mitochondrial/Multigenic
X-linked dominant
X-linked recessive
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Autoimmune lymphoproliferative syndrome (ALPS) is characterized by dysregulation of the immune system due to an inability to regulate lymphocyte homeostasis through the process of lymphocyte apoptosis
5 Facts you should know
FACT
A disorder which is caused by defective lymphocyte homeostasis
FACT
This results in the overproduction of lymphocytes, which build up and cause enlargement of the lymph nodes, liver, and spleen
FACT
ALPS can also cause anemia, thrombocytopenia, and neutropenia
FACT
Affected individuals have an increased risk of various autoimmune diseases
FACT
Patients are also at increased risk for both Hodgkin's and non-Hodgkin's lymphoma
Interest over time
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Common signs & symptoms
Chronic noninfectious lymphadenopathy
Splenomegaly
Increased spleen size
Abnormal serum interleukin level
Autoimmune hemolytic anemia
Autoimmune thrombocytopenia
Bruising susceptibility
Current treatments
Immunosuppressive Therapy
Immunosuppressive medications, such as corticosteroids (e.g., prednisone) and immunosuppressants (e.g., mycophenolate mofetil, sirolimus), are often used to manage ALPS symptoms by suppressing the overactive immune response. These medications can help reduce lymphoproliferation, autoimmune cytopenias, and other disease manifestations
Splenectomy
Splenectomy (surgical removal of the spleen) is another treatment option for ALPS, particularly for patients who do not respond well to immunosuppressive therapy or who have severe cytopenias. Splenectomy can help alleviate symptoms by reducing the destruction of blood cells and decreasing lymphoproliferation.