ABSTRACT

Disorders of immunologic deficiency may be viewed as specific defects of cellular maturation, immunologic recognition, immune regulation, or effector function which together are essential for a normal immune response. This chapter deals with disorders of immunologic deficiency including experimental, primary, and secondary disorders in which suppressor cell function has been identified. A more inviting hypothesis is that the suppressor defect observed in patients with X-Linked Agammaglobulinemia is similar to the infectious agammaglobulinemia of birds described originally by Blaese. Common Variable Hypogammaglobulinemia encompasses a spectrum of disorders all sharing a varying degree of panhypogammaglobulinemia and variable age of onset. Selective IgA deficiency is the most common immune deficiency of man occurring in about 1 of 600 in the general population. Unlike the immunodeficiencies associated with thymoma and multiple myeloma which are primarily humoral, the immunodeficiency frequently observed in patients with Hodgkin's is primarily cellular with their humoral immunity apparently intact.