Intestinal, Salivary, and Tonsillar IgA and J‐Chain Production in a Patient with Severe Deficiency of Serum IgA

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Abstract
An 18‐year‐old man with tendency to respiratory infections had a serum IgA level of only 2% of normal whereas his salivary IgA amounted to 50% of the lower normal concentration range. Moreover, both the rectal and jejunal IgA‐producing cell populations were of normal size. Nevertheless, a relative increase of salivary IgM and a distinctly raised number of IgM‐producing cells in jejunal mucosa indicated an imbalance in his secretory immune system. This possibility was supported by the presence of an excess of J chain in most of his intestinal IgA immunocytes, probably reflecting a reduced synthetic rate of IgA. The number of tonsillar IgA‐producing cells was only slightly below the normal range: most of them lacked J chain, as normal, and could thus be a source of his serum IgA, which was mainly monomeric. A marked deficiency of IgA‐producing cells in his hone marrow supported the notion that this tissue site normally is the major source of monomeric IgA. This study suggests that a generally defective IgA system may he topically activated owing to the persistent antigenic and mitogenic load on mucosa‐associated lymphoid tissues. Our findings are not consistent with a general regulative compartmentalization of monomer‐ and dimer‐producing IgA immunocyte populations.