Expansion of large granular lymphocyte subsets in Wiskott-Aldrich syndrome.
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.
Plebani
A
A
Istituto G. Gaslini, Università degli Studi di Genova, Italy.
Airò
P
P
Brugnoni
D
D
Lebowitz
M
M
Cattaneo
R
R
Monafo
V
V
Meini
A
A
Notarangelo
L D
LD
Duse
M
M
Ugazio
A G
AG
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