Hypogammaglobulinemia with Nodular Lymphoid Hyperplasia of the Small Intestine

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  • ARIKAWA Kazumi
    The Department of Medicine, School of Medicine, Keio University
  • Asakura Hitoshi
    The Department of Medicine, School of Medicine, Keio University
  • ICHIKAWA Yoichi
    The Department of Medicine, School of Medicine, Keio University
  • ABE Tohru
    The Department of Medicine, School of Medicine, Keio University
  • HOMMA Mitsuo
    The Department of Medicine, School of Medicine, Keio University

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  • Hypogammaglobulinemia with Nodular Lymp

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Abstract

A case of hypogammaglobulinemia with nodular lymphoid hyperplasia of the small intestine was presented with its immunological analysis. A peroral biopsy of the proximal jejunum demonstrated enlarged lymphoid follicles with large germinal centers. Only a few plasma cells were observed. Serum IgG and IgM were markedly reduced, IgA, IgD and IgM could not be traced. Secretory IgA was absent in saliva, while normal amount of secretory component was present. Peripheral B-lymphocytes (B-cells) enumerated by surface immunoglobulin receptors were rather increased while B-cells determined by C3 receptors were markedly decreased. Peripheral T-lymphocytes measured by sheep red blood cell rosette formation were increased in number, but their function was depressed as judged by the response to PHA. The pathogenesis of this disorder was discussed, reviewing 27 cases of this syndrome so far reported.

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