Haematologica
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Haematologica, Vol 80, Issue 6, 521-525
Copyright © 1995 by Ferrata Storti Foundation

Case Reports

Expansion of large granular lymphocyte subsets in Wiskott-Aldrich syndrome

A Plebani, P Airo, D Brugnoni, M Lebowitz, R Cattaneo, V Monafo, A Meini, LD Notarangelo, M Duse, and AG Ugazio

Istituto G. Gaslini, Universita degli Studi di Genova, Italy.

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.


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PNAS, August 20, 2002; 99(17): 11351 - 11356.
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Copyright © 1995 by the Ferrata Storti Foundation.