The Primary Immunodeficiencies
- 17 August 1995
- journal article
- review article
- Published by Massachusetts Medical Society in New England Journal of Medicine
- Vol. 333 (7) , 431-440
- https://doi.org/10.1056/nejm199508173330707
Abstract
The primary immunodeficiency disorders reflect abnormalities in the development and maturation of cells of the immune system. These defects result in an increased susceptibility to infection; recurrent pyogenic infections occur with defects of humoral immunity, and opportunistic infections with defects of cell-mediated immunity. These two broad categories of illness correspond roughly to defects in the two principal types of immunocompetent cells, B lymphocytes and T lymphocytes. Defective development of B cells results in abnormalities in humoral immunity, whereas defects in the development of T cells cause problems with cellular immunity.When pathogens are taken up by macrophages or dendritic cells, . . .Keywords
This publication has 104 references indexed in Scilit:
- Unique mutations of Bruton's tyrosine kinase in fourteen unrelated X-linked agammaglobulinemia familiesHuman Molecular Genetics, 1994
- X-linked thrombocytopenia and Wiskott-Aldrich syndrome: similar regional assignment but distinct X-inactivation pattern in carriersAnnals of Hematology, 1991
- Severe Combined Immunodeficiency Due to a Specific Defect in the Production of Interleukin-2New England Journal of Medicine, 1990
- An Immunodeficiency Characterized by Defective Signal Transduction in T LymphocytesNew England Journal of Medicine, 1989
- Regulation of Genes for HLA Class II Antigens in Cell Lines from Patients with Severe Combined ImmunodeficiencyNew England Journal of Medicine, 1988
- Treatment of Adenosine Deaminase Deficiency with Polyethylene Glycol–Modified Adenosine DeaminaseNew England Journal of Medicine, 1987
- Carrier Detection in X-Linked Agammaglobulinemia by Analysis of X-Chromosome InactivationNew England Journal of Medicine, 1987
- Expression of the Gene Defect in X-Linked AgammaglobulinemiaNew England Journal of Medicine, 1986
- Evidence for a Defect in Switch T Cells in Patients with Immunodeficiency and Hyperimmunoglobulinemia MNew England Journal of Medicine, 1986
- Complete Correction of the Wiskott-Aldrich Syndrome by Allogeneic Bone-Marrow TransplantationNew England Journal of Medicine, 1978