Abstract
IgA deficiency is the most common humoral immune defect. We examined blood lymphocytes from 4 patients with Ataxia Telangiectasia and 6 with common IgA deficiency. All had absent serum IgA but .5–2% sIgA+ circulating B cells. PWM and EBV were used for the stimulation of these cells either in high density cultures (106 cells/well) or in low density, limiting dilution cultures (1–104 cells/well), which restrict cell:cell interactions. IgG, A and M secreted into culture supernates was measured by RIA and ELISA after 8 d (PWM) or 4 wk of culture (EBV). Cells from patients failed to produce IgA in high density cultures. In EBV stimulated limiting dilution cultures, IgA producing cells were detected in all patients. The frequency of IgA-committed, EBV transformable cells was normal in patients with uncomplicated IgA deficiency (approximately 1/100 B cells). However, in patients with AT, the frequency of IgA producing cells was up to 40 times lower. Our experiments demonstrate the presence of IgA producing cells in all patients with IgA deficiency studied. No evidence for IgA specific B cell defects could be delineated in common IgA deficiency. In AT, abnormalities of B cell differentiation may lead to the development of a smaller, IgA-committed B cell compartment.
Supported by the Arthritis Society, MRC and NCI of Canada.
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Stein, L., Roifman, C., Levi, S. et al. 1028 FUNCTIONAL ANALYSIS OF IgA PRODUCING CELLS FROM PATIENTS WITH SELECTIVE IgA DEFICIENCY. Pediatr Res 19, 282 (1985). https://doi.org/10.1203/00006450-198504000-01058
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DOI: https://doi.org/10.1203/00006450-198504000-01058