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 CD8(dim) 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.
Expansion of large granular lymphocyte subsets in Wiskott-Aldrich syndrome / A., Plebani; P., Airo; D., Brugnoni; M., Lebowitz; R., Cattaneo; V., Monafo; A., Meini; L. D., Notarangelo; Duse, Marzia; A. G., Ugazio. - In: HAEMATOLOGICA. - ISSN 0390-6078. - STAMPA. - 80:6(1995), pp. 521-525.
Expansion of large granular lymphocyte subsets in Wiskott-Aldrich syndrome
DUSE, MARZIA;
1995
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 CD8(dim) 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.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
