Document Detail

Hypogammaglobulinemia with hyper-IgM, severe T-cell defect, and abnormal recirculation of OKT4 lymphocytes in a girl with chronic lymphadenopathy.
MedLine Citation:
PMID:  3079686     Owner:  NLM     Status:  MEDLINE    
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.
M Fiorilli; G Russo; R Paganelli; C Papetti; M Carbonari; M Crescenzi; M Calvani; I Quinti; F Aiuti
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Publication Detail:
Type:  Case Reports; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Clinical immunology and immunopathology     Volume:  38     ISSN:  0090-1229     ISO Abbreviation:  Clin. Immunol. Immunopathol.     Publication Date:  1986 Feb 
Date Detail:
Created Date:  1986-02-12     Completed Date:  1986-02-12     Revised Date:  2008-11-21    
Medline Journal Info:
Nlm Unique ID:  0356637     Medline TA:  Clin Immunol Immunopathol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  256-64     Citation Subset:  IM; X    
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MeSH Terms
Agammaglobulinemia / blood,  immunology*
Antibodies, Monoclonal
B-Lymphocytes / metabolism
Chronic Disease
Hypergammaglobulinemia / blood,  immunology*
Immunoglobulin M / biosynthesis*
Interferon-gamma / biosynthesis
Lymph Nodes / immunology
Lymphoproliferative Disorders / blood,  immunology*
T-Lymphocytes / classification,  immunology*,  physiology
Reg. No./Substance:
0/Antibodies, Monoclonal; 0/Immunoglobulin M; 82115-62-6/Interferon-gamma

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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