obsolete ALPS-associated lymphoma

Based on the provided context, it appears that Autoimmune Lymphoproliferative Syndrome (ALPS)-associated lymphoma is a type of cancer that can occur in individuals with ALPS.

Characteristics:

• Age of onset: Typically presents at a median age of 2.7 to 3 years [13].
• Clinical manifestations: May be accompanied by lymphadenopathy, splenomegaly, and increased risk of autoimmune disease [15].
• Lymphocyte subsets: Monoclonal expansion of malignant lymphocytes can occur, which may help distinguish benign from malignant disease [11].

Description:

ALPS-associated lymphoma is a rare and complex condition that requires early recognition and diagnosis for successful management and treatment. The disease often presents with an enlarged spleen and lymph nodes containing an abnormally high DNT population, autoimmune cytopenias driven by autoantibody production, and an increased risk of lymphoma [4].

Key features:

• Lymphadenopathy: Enlarged lymph nodes are a common feature of ALPS-associated lymphoma.
• Splenomegaly: An enlarged spleen is often present in individuals with this condition.
• Autoimmune cytopenias: Autoantibody production can lead to autoimmune cytopenias, which affect the hematopoietic cell lines.

References:

[4] AL Snow (2010) - Features of ALPS include an enlarged spleen and lymph nodes containing an abnormally high DNT population, autoimmune cytopenias driven by autoantibody production [8]. [11] Since progression to lymphoma is associated with monoclonal expansion of malignant lymphocytes, occasionally testing for clonality in lymphocyte subsets can help distinguish benign from malignant disease. [13] In the two largest cohorts of patients with ALPS, a French cohort and a cohort from the National Institutes of Health (NIH), disease onset was most commonly marked by lymphoproliferation with generalized adenopathy and hepatosplenomegaly at a median age of 2.7 to 3 years. [15] Autoimmune lymphoproliferative syndrome (ALPS) is characterized by immune dysregulation due to a defect in lymphocyte apoptosis, which may lead to the development of ALPS-associated lymphoma.