Hyper IgM immunodeficiency. A primary dysfunction of B lymphocyte isotype switching.
@article{Levitt1983HyperII,
title={Hyper IgM immunodeficiency. A primary dysfunction of B lymphocyte isotype switching.},
author={Daniel Levitt and Patricia L. Haber and Kevin Rich and Max D. Cooper},
journal={The Journal of clinical investigation},
year={1983},
volume={72 5},
pages={
1650-7
}
}Immunological evaluations (lymphocyte markers, B cell differentiation, T cell function) were performed on peripheral blood mononuclear cells from four individuals with hyper IgM immunodeficiency. Number, proportion, and proliferation of T lymphocytes and T lymphocyte subpopulations were relatively normal in affected individuals. The percentage and number of B cells expressing surface IgM and IgD were either normal or elevated in both blood and lymph nodes. However, surface IgG- and IgA-bearing…
76 Citations
Hyper-IgM immunodeficiency syndrome: Influence of lymphokines onin vitro maturation of peripheral B cells
- Medicine, BiologyJournal of Clinical Immunology
- 2004
The hyper-IgM immunodeficiency syndrome is associated with a defect in lymphokine-dependent maturation of B lymphocytes, although the T- or the B-cell origin of the defect still cannot be determined.
Expansion of a suppressor T-cell population associated with the hyper-IgM syndrome and generalized lymphadenopathy.
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- 1991
Human malignant T cells capable of inducing an immunoglobulin class switch
- Medicine, BiologyThe Journal of experimental medicine
- 1985
Evidence is presented for the existence of a "switch" T cell derived from a patient with mycosis fungoides/Sezary's syndrome, which appeared to induce an Ig class switch from IgM to IgG and IgA, when co-cultured with mu+ tonsillar B cells.
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- Biology, MedicineClinical and diagnostic laboratory immunology
- 1995
The data suggest that the B cells of patients with HIGM1 possess an additional defect which prevents Ig isotype switching in response to T cell-independent mitogens, not located in the membrane receptors or within the membrane enzymes.
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- Medicine, BiologyJournal of Clinical Immunology
- 2004
We investigated the prevalence of phenotypically immature IgG B cells (i.e., coexpressing surface IgG and IgM) in the peripheral blood of 12 patients with hypogammaglobulinemia and in normal…
Decreased expression of the ligand for CD40 in newborn lymphocytes
- Biology, MedicineEuropean journal of immunology
- 1994
Results indicate that decreased expression of CD40 ligand in newborn lymphocytes may be the underlying cause of deficient immunoglobulin isotype switching in newborns.
Evidence for a defect in "switch" T cells in patients with immunodeficiency and hyperimmunoglobulinemia M.
- Medicine, BiologyThe New England journal of medicine
- 1986
B cells from patients with hyperimmunoglobulinemia M may not be abnormal and that, at least in some patients with this syndrome, a defect in switch T cells may be pathogenic, according to analysis of the immunoglobulin heavy-chain genes.
The role of CD40 in the immune response.
- Biology, Medicine
- 1998
Results confirm the crucial role of the CD40-CD40L interaction in humoral immunity and derive an animal model for HIGMl, which is characterized by the inability of B cells to undergo immunoglobulin isotype switching.
Hypogammaglobulinemia with hyper-IgM, severe T-cell defect, and abnormal recirculation of OKT4 lymphocytes in a girl with chronic lymphadenopathy.
- Medicine, BiologyClinical immunology and immunopathology
- 1986
CD40 ligand gene defects responsible for X-linked hyper-IgM syndrome
- Biology, MedicineScience
- 1993
Abnormalities in the CD40L gene were associated with an X-linked immunodeficiency in humans [hyper-IgM (immunoglobulin M) syndrome], characterized by elevated concentrations of serum IgM and decreased amounts of all other isotypes.
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