101. Expansion of large granular lymphocyte subsets in Wiskott-Aldrich syndrome
- Author
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Plebani, A., Airò, P., Duilio Brugnoni, Lebowitz, M., Cattaneo, R., Monafo, V., Meini, A., Notarangelo, L. D., Duse, M., and Ugazio, A. G.
- Subjects
Arthritis, Rheumatoid ,Male ,lgl ,wiskott-aldrich syndrome ,Humans ,Child ,Lymphocyte Subsets ,Cell Size - 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.