Description of the disease * EnglishPolish Pobierz sekcję do PDF Definition 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. Disease data Classification Disease Synonyms B-cell expansion with NF-kB and T-cell anergy disease B-cell expansion with NF-kB and T-cell anergy disease ORPHA code 464336 OMIM code 616452 ICD10 code D81.8 ICD11 code - *Soruce Extended description of the disease Pobierz sekcję do PDF No additional description. Orphanet - interntowa baza danych dotyczących rzadkich chorób i sierochych leków. ©INSERM 1999 - Dostępna na stronie www.orphanet.pl