PRIMARY IMMUNODEFICIENCY DISEASES ASSOCIATED WITH INTESTINAL NODULAR LYMPHOID HYPERPLASIA, WITH SPECIAL REFERENCE TO UNIQUE FEATURES OF ILLNESS AND PATHOGENESIS

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Department of Internal Medicine, Hyogo Prefectural Amagasaki Hospital, Hyogo In 1966, Hermans et al. noted a complex of abnormalities in primary a-or dysgammaglobulinemia associated with nodular lymphoid hyperplasia (NLH) of the small intestine, which led its classification as a separate group (Hermans' syndrome). Since the WHO classification of primary immunodeficiency diseases has been proposed in 1971, this syndrome is lumped under the title of common variable immunodeficiency (CVID) without making separation from the others of CVID. Here, we have reevaluated it mainly through the investigation of our two typical cases and of 62 cases (including ours) reported in the literature as to whether primary immunodeficiency diseases associated with NLH can be separated as a nosologic group. We have found that these cases have clinical as well as immunologic features in common with each other. As a consequence of the foregoing, it is reasonable to separate this syndrome from the others of CVID. It seems likely that NLH represents an "ineffective" proliferation of B cells which can not differentiate into immunoglobulin-producing plasma cells. Moreover, the following two mechanisms seem to be implicated with the development of NLH: (1) loss of feed back inhibition of B cell proliferation by antibodies, and (2) prolonged stimulation of gut-associated lymphoid tissues by antigens absorbed excessively due to lack of secretory IgA. Finally, it has been suggested that there are certain developmental abnormalities of the gastrointestinal mucosa in this syndrome.
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PRIMARY IMMUNODEFICIENCY DISEASES ASSOCIATED WITH INTESTINAL NODULAR LYMPHOID HYPERPLASIA, WITH SPECIAL REFERENCE TO UNIQUE FEATURES OF ILLNESS AND PATHOGENESIS

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