BENTA sykdom
Engelsk navn: BENTA disease
Engelske synonym: B-cell expansion with NF-kB and T-cell anergy disease
Definisjon
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.
Fra Orphanet
ORPHA: 464336
Klassifiseringsnivå: Disorder
ICD-10: D81.8
Mer informasjon
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