HYPER IGM IMMUNODEFICIENCY - A PRIMARY DYSFUNCTION OF LYMPHOCYTE-B ISOTYPE SWITCHING

HYPER IGM IMMUNODEFICIENCY - A PRIMARY DYSFUNCTION OF LYMPHOCYTE-B ISOTYPE SWITCHING
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DOI:
10.1172/jci111124
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发表时间:
1983-01-01
影响因子:
15.9
通讯作者:
COOPER, MD
COOPER, MD
中科院分区:
医学1区
文献类型:
--
作者:
LEVITT, D;HABER, P;COOPER, MD

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Immunological evaluations (lymphocyte markers, B cell differentiation, T cell function) were performed on peripheral blood mononuclear cells from 4 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. Surface IgG- and IgA-bearing B lymphocytes were completely absent. In vitro stimulation of blood lymphocytes with both T cell-dependent and T cell-independent polyclonal B cell activators resulted in normal numbers of IgM plasma cells and IgM secretion in cultures, but failed to induce any IgG- or IgA-producing cells. This failure of isotype switching was intrinsic to the B cell population and did not involve aberrant T cell help or suppression. Individuals with this disorder possess an intrinsic B cell dysfunction that is not related to abnormal T cell regulation.