Heterogeneity of hypogammaglobulinemia

Abstract In 1952, Ogden Bruton described the first patient with recurrent infections and hypogammaglobulinemia [1]. For many years thereafter, hypogammaglobulinemia (or agammaglobulinemia) was believed to result from a deficiency (or absence) of the antibody‐producing cells, or “B” cell line of the lymphoid system. With increased recognition of functional subpopulations of the lymphoid system, however, it has become apparent that a variety of mechanisms may result in the phenotype of hypogammaglobulinemia. Since these entities may be clinically indistinguishable, it is important to understand the various categories of disease which may be involved. In this concise review, these areas are briefly summarized, More complete reviews of the subject (including acquired forms of immunodeficiency) are available [2, 3].