title: Expansion of large granular lymphocyte subsets in Wiskott-Aldrich syndrome.
abstract: We describe a 9-year-old boy with Wiskott-Aldrich syndrome and IgM-rheumatoid factor-positive arthritis who presented expansion of two distinct subsets (one CD8dim and the other CD8-) of large granular lymphocytes. Natural killer activity against the K-562 cell line was absent. An increased percentage of CD5+ B cells was also observed. Since patients with Wiskott-Aldrich syndrome are at risk of developing autoimmune disorders - conditions in which increased CD5+ B cells have been observed - the high percentage of CD5+ B cells together with the presence of IgM-rheumatoid factor and anti-platelet antibodies may represent an early manifestation of an autoimmune process. The possible relationship between CD5+ B cells and large granular lymphocyte expansion is discussed.
authors.affiliation: Istituto G. Gaslini, Università degli Studi di Genova, Italy.
authors.forename: A
authors.initials: A
authors.lastname: Plebani
authors.forename: P
authors.initials: P
authors.lastname: Airò
authors.forename: D
authors.initials: D
authors.lastname: Brugnoni
authors.forename: M
authors.initials: M
authors.lastname: Lebowitz
authors.forename: R
authors.initials: R
authors.lastname: Cattaneo
authors.forename: V
authors.initials: V
authors.lastname: Monafo
authors.forename: A
authors.initials: A
authors.lastname: Meini
authors.forename: L D
authors.initials: LD
authors.lastname: Notarangelo
authors.forename: M
authors.initials: M
authors.lastname: Duse
authors.forename: A G
authors.initials: AG
authors.lastname: Ugazio
mesh_terms: D001172:Arthritis, Rheumatoid / Q000276:immunology* / Q000473:pathology; D048429:Cell Size; D002648:Child; D006801:Humans; D016131:Lymphocyte Subsets / Q000276:immunology* / Q000473:pathology; D008297:Male; D014923:Wiskott-Aldrich Syndrome / Q000276:immunology* / Q000473:pathology