BENTA disease

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BENTA disease

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.

Reference: Access aggregated data from Orphanet at Orphadata.

Orphadata: Free access data from Orphanet. © INSERM 1999. Available on Data version March 2024

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Clinical Trials

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