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Review Article
Medical Progress
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Volume 333:431-440 August 17, 1995 Number 7
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The Primary Immunodeficiencies
Fred S. Rosen, M.D., Max D. Cooper, M.D., and Ralph J.P. Wedgwood, M.D.

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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, . . . [Full Text of this Article]

X-Linked Agammaglobulinemia

Hyper-IgM Syndrome

Common Variable Immunodeficiency

Severe Combined Immunodeficiency

Defects in the Expression of the MHC

The Wiskott–Aldrich Syndrome


Source Information

From the Departments of Pediatrics, Harvard Medical School, Boston (F.S.R.), the University of Alabama School of Medicine, Birmingham (M.D.C.), and the University of Washington School of Medicine, Seattle (R.J.P.W.).

Address reprint requests to Dr. Rosen at the Center for Blood Research, 800 Huntington Ave., Boston, MA 02115.

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