Inherited Dysgammaglobulinemia of Chickens

Abstract

Disorders of the immune system leading to hypogammaglobulinemia are considered to be either primary defects in stem cell precursors of B lymphocytes or disorders in the maturation of B cells. In the X-linked form of immunodeficiency B cells are usually absent; however, patients with the form of immunodeficiency referred to as varied immunodeficiency (VID) have normal or slightly below-normal numbers of circulating B cells (Dickler et al, 1974; Geha et al, 1974; Cooper et al, 1975). In VID, reduced immunoglobulin (Ig) results from defects that may occur at various stages of B-cell maturation—from impaired differentiation of pre-B cells to defective terminal differentiation (Pearl et al, 1979). The failure of B cells to mature into Ig-secreting cells has been associated with different abnormalities, such as excessive suppressor-cell activity (Waldmann et al, 1974, 1976; Siegal et al, 1976, 1978), defective helper-cell activity (De La Concha et al, 1977; Siegal et al, 1978), presence of serum and tissue-fluid inhibitors (Geha et al, 1974; Waldmann et al, 1976), and inherent B-cell defects (Geha et al, 1974; Ciccimarra et al, 1976; De La Concha et al, 1977). Clearly, VID is heterogeneous with respect to its phenotypic features, its mechanism of defective B-cell synthesis, and its inheritance patterns.