Synonyms: B-cell expansion with NF-kB and T-cell anergy disease
A rare primary immunodeficiency characterized by infantile onset of generalized lymphadenopathy splenomegaly and lymphocytosis with excessive polyclonal expansion of B-cells. Patients present recurrent infections and impaired T-cell and antibody responses while overt autoimmune manifestations are usually absent. Occurrence of B-cell malignancy later in life has been reported.
Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview.
Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http://www.orphadata.org. Data version March 2024
Newly diagnosed with
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