Primary Immunodeficiency Syndromes: Challenges and Progress in Immune Health

Primary immunodeficiency syndromes are genetically determined immunodeficiencies with immune and nonimmune defects. Nonimmune manifestations are often more easily recognized than those of the immunodeficiency. Examples are ataxia-telangiectasia, cartilage-hair hypoplasia, DiGeorge syndrome, hyper-IgE syndrome, and Wiskott-Aldrich syndrome. Despite the presence of immunodeficiencies, some patients also develop autoimmune disorders.