Hypogammaglobulinemia with hyper-IgM, severe T-cell defect, and abnormal recirculation of OKT4 lymphocytes in a girl with chronic lymphadenopathy. NLM AIDSLINE Important note: Information in this article was accurate in 1986. The state of the art may have changed since the publication date.

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Hypogammaglobulinemia with hyper-IgM, severe T-cell defect, and abnormal recirculation of OKT4 lymphocytes in a girl with chronic lymphadenopathy.

Clin Immunol Immunopathol. 1986 Feb;38(2):256-64. Unique Identifier : AIDSLINE MED/86080250
Fiorilli M; Russo G; Paganelli R; Papetti C; Carbonari M; Crescenzi M; Calvani M; Quinti I; Aiuti F

Abstract: We describe here one 8-year-old girl with an unusual form of immunodeficiency, characterized by hypogammaglobulinemia with hyper-IgM, severe T-cell defect, and chronic lymphadenopathy. Patient's B cells failed to produce IgG or IgA in vitro following stimulation with either pokeweed mitogen or Epstein-Barr virus, suggesting an intrinsic B-cell defect. Abnormal T-cell function was demonstrated by impaired in vivo delayed type hypersensitivity, reduction of mitogen-induced proliferation and interleukin 2 production, reduction of interferon-gamma production, and marked decrease of circulating OKT4+ cells. The latter cells were found in normal proportion in the patient's lymph node tissue. This finding suggests that the decrease of OKT4+ cells in peripheral blood was due to the abnormal recirculation of these cells. The identity of this syndrome with the infantile form of the acquired immunodeficiency syndrome was apparently ruled out by the failure to demonstrate HTLV-III-related sequences in patient's lymphocytes or virus-specific serum antibodies.
Keywords: Agammaglobulinemia/BLOOD/*IMMUNOLOGY Antibodies, Monoclonal B-Lymphocytes/METABOLISM Case Report Child Chronic Disease Female Human Hypergammaglobulinemia/BLOOD/*IMMUNOLOGY IgM/*BIOSYNTHESIS Interferon Type II/BIOSYNTHESIS Lymph Nodes/IMMUNOLOGY Lymphoproliferative Disorders/BLOOD/*IMMUNOLOGY Phenotype Support, Non-U.S. Gov't T-Lymphocytes/CLASSIFICATION/*IMMUNOLOGY/PHYSIOLOGY JOURNAL ARTICLE

KWDagammaglobulinemia/blood/KWDimmunologyantibodies,monoclonalb-lymphocytes/metabolismcasereportchildchronicdiseasefemalehumanhypergammaglobulinemia/blood/KWDimmunologyigm/KWDbiosynthesisinterferontypeii/biosynthesislymphnodes/immunologylymphoproliferativedisorders/blood/KWDimmunologyphenotypesupport,non-uKWDsKWDgov'tt-lymphocytes/classification/KWDimmunology/physiologyjournalarticle
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M8640109

Copyright © 1986 - National Library of Medicine. Reproduced under license with the National Library of Medicine, Bethesda, MD.

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