EXPANSION OF LARGE GRANULAR LYMPHOCYTE SUBSETS IN WISKOTT-ALDRICH SYNDROME

We describe a 9-year-old boy with Wiskott-Aldrich syndrome and IgM-rhe umatoid factor-positive arthritis who presented expansion of two disti nct subsets (one CD8(dim) and the other CD8(-)) of large granular lymp hocytes. Natural killer activity against the K-562 cell line was absen t. An increased percentage of CD5(+) B cells was also observed. Since patients with Wiskott-Aldrich syndrome are at risk of developing autoi mmune disorders - conditions in which increased CD5(+) B cells have be en observed - the high percentage of CD5(+) B cells together with the presence of IgM-rheumatoid factor and anti-platelet antibodies may rep resent an early manifestation of an autoimmune process. The possible r elationship between CD5(+) B cells and large granular lymphocyte expan sion is discussed.