SUMMARYExpression of the ;/^ Tcell receptor (TCR) on CD3' intraepithelial lymphocytes (lELs) was studied by two-eolour immunofluorescence in duodenal tissue sections from healthy (n = 6) or infeetionprone{/) = 7) subjects wilh seleetive IgA deficiency (IgAD), and subjects (« = 4) with combined IgAD and IgG subclass deficiency, TCRy/VJ* IEL proportions in selective IgAD subjects (median 6 .3"'n, range I 0-41'%.) and in those wilh combined deficiency (median 4-5"A>, range l'2-33%) were well within the range (0 3 38'^ii) for hisiologically normal controls (n=II), but ihe healthy IgAD subgroup tended to show raised TCR)7'(>' IEL proportions (median 13-6'M.) compared with the other two subgroups. Also the number of TCRy/Vi^ lELs per intestinal length iinii was relatively high (median I3-9/mm) in ihe healthy IgAD subjects, and significantly raised (/'<0 ()3) compared with controls (median 3-2 'mm). Paired staining revealed that most TCRy/i'J' lELs in bolh seleetive igAD (98%) and combined deficiency (99';..) were CDH . and a large fraction (median ^4"/.! and 63"^, respectively) expressed the V(5l,/J(5l-encoded epitope. The total number of CD3^ IELs (mostly CD8 ') was simitar lo controls. IgAD subject.s. and especially the healthy subgroup, had significanlly increased serum eoncenlraiions of soluble CD8 (P<0 0002), ncoplerin (/'<() ()05), and 2-microglobulin (/'<0007), which was similar to our previous observations in conmion variable immunodeficiency, and probably reflected stimulation of cell-media ted immunity. In addition, the inereased TCRylS^ IELs might reflect a component of compensatory surface proteelion in the healthy IgAD subgroup.