Immune regulation: what immunodeficiency disease has taught us.

"Immune regulation: what immunodeficiency disease has taught us" is reviewed by discussing three immuno-deficiency disorders. Hypogammaglobulinemia, the first documented primary immunodeficiency disorder, has a well defined and uniform clinical presentation which reflects a variety of underlying abnormalities involving the B cell, T cell, and monocyte. X-linked hypogammaglobulinemia, transient hypogammaglobulinemia of infancy common variable immunodeficiency, and their pathogenesis are discussed. Combined immunodeficiency with adenosine deaminase (ADA) deficiency first led to the now accepted concept that a biochemical abnormality may result in immunodeficiency. The clinical presentation, possible biochemical abnormalities resulting in the observed immunodeficiency, relative selectivity of the defect for the immune system, and potential applications of knowledge gained from the study of ADA deficiency are presented. Acquired immunodeficiency (AIDS) has resulted in the concept that a virus is cytopathic for a specific population of T cells and that this, at least in part, results in the immunodeficiency seen in AIDS.