Introduction: Cellular Aspects of Immune Deficiency Diseases Involving B Cells

Abstract

The discovery in 1952 of agammaglobulinemia in a boy having repeated bacterial infections was shortly followed by the demonstration that a scarcity of plasma cells and of lymphocytes in primary follicles and germinal centers was the cellular basis for the antibody deficiency (1). The succeeding two decades have witnessed the unfolding of a challenging array of immunodeficiencies in man and other species. Some of these have been reproduced experimentally and the interplay between natural and contrived models of immunodeficiency has contributed to an increased understanding of the cellular basis for development and function of the immune system. A prime example of the benefits accrued from the interplay is the knowledge that the immune system develops from hemopoietic stem cells directed along thymus (T) and bursal (B) avenues of lymphoid differentiation (2). Thus, immunodeficiencies can be broadly classified as errors of differentiation involving T cells, B cells, or stem cells (3). Beyond their theoretical importance, these concepts have led to successful repair of immunodeficiency diseases in a few individuals by bone marrow or thymus transplants, examples of which will be presented in this session.