title: Reduced Number of CD8+ Cells in Tonsillar Germinal Centres in Children with the Periodic Fever, Aphthous Stomatitis, Pharyngitis and Cervical Adenitis Syndrome.
abstract: The syndrome of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) is an autoinflammatory disorder of unknown aetiology. Tonsillectomy may cause a prompt resolution of the syndrome. The aim was to study the histologic and immunological aspects of the palatine tonsils in PFAPA, to help understand the pathophysiology of the syndrome. Tonsils from children with PFAPA (n = 11) and children with tonsillar hypertrophy (n = 16) were evaluated histologically after haematoxylin and eosin staining. The number of different cell types was identified immunohistochemically by cluster of differentiation (CD) markers: CD3 (T cells), CD4 (T helper cells), CD8 (cytotoxic T cells), CD15 (neutrophils), CD20 (B cells), CD45 (all leucocytes), CD57 (NK cells) and CD163 (monocytes and macrophages). Tonsils from children with PFAPA showed reactive lymphoid hyperplasia dominated by well-developed germinal centres with many tingible body macrophages. The histologic findings were unspecific, and a similar morphologic appearance was also found in the tonsils from controls. The number of CD8+ cells in germinal centres differed between children with PFAPA [median 9 cells (quartiles: 5, 15)] and controls [18 cells (12, 33) (P = 0.001)] and between children with PFAPA with (median 14 cells; 9, 16) and without (4 cells; 3, 8) aphthous stomatitis (P = 0.015). For the other cell types, no differences in germinal centres were found between children with PFAPA and controls. In conclusion, a lower number of CD8+ cells were found in germinal centres of tonsils in children with PFAPA compared to controls, which may be a feature linked to the aetiology of the syndrome.
authors.affiliation: Department of Pediatrics, Stavanger University Hospital, Stavanger, Norway.
authors.forename: J
authors.initials: J
authors.lastname: Førsvoll
authors.affiliation: Department of Pathology, Stavanger University Hospital, Stavanger, Norway.
authors.forename: E A M
authors.initials: EA
authors.lastname: Janssen
authors.affiliation: Department of Otolaryngology, Stavanger University Hospital, Stavanger, Norway.
authors.forename: I
authors.initials: I
authors.lastname: Møller
authors.affiliation: Department of Otolaryngology, Stavanger University Hospital, Stavanger, Norway.
authors.forename: N
authors.initials: N
authors.lastname: Wathne
authors.affiliation: Department of Pathology, Stavanger University Hospital, Stavanger, Norway.
authors.forename: I
authors.initials: I
authors.lastname: Skaland
authors.affiliation: Department of Pathology, Stavanger University Hospital, Stavanger, Norway.
authors.forename: J
authors.initials: J
authors.lastname: Klos
authors.affiliation: Department of Clinical Science, University of Bergen, Bergen, Norway.|Department of Immunology and Transfusion Medicine, Haukeland University Hospital, Bergen, Norway.
authors.forename: E K
authors.initials: EK
authors.lastname: Kristoffersen
authors.affiliation: Department of Pediatrics, Stavanger University Hospital, Stavanger, Norway.|Department of Clinical Science, University of Bergen, Bergen, Norway.
authors.forename: K
authors.initials: K
authors.lastname: Øymar
mesh_terms: D015496:CD4-Positive T-Lymphocytes / Q000276:immunology; D018414:CD8-Positive T-Lymphocytes / Q000276:immunology*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005334:Fever / Q000276:immunology*; D018858:Germinal Center / Q000166:cytology / Q000276:immunology*; D056660:Hereditary Autoinflammatory Diseases / Q000276:immunology*; D006801:Humans; D007694:Killer Cells, Natural / Q000276:immunology; D008199:Lymphadenitis / Q000276:immunology*; D018655:Lymphocyte Count; D008264:Macrophages / Q000276:immunology; D008297:Male; D009000:Monocytes / Q000276:immunology; D009504:Neutrophils / Q000276:immunology; D014066:Palatine Tonsil / Q000276:immunology* / Q000601:surgery; D010612:Pharyngitis / Q000276:immunology*; D013281:Stomatitis, Aphthous / Q000276:immunology*; D013577:Syndrome; D014068:Tonsillectomy