Primary antibody deficiencies represent the most frequent
genetic diseases of the immune system and the first to be recognized along immunology history. The
antibodies were recognized as part of the humoral immune system long ago, and after
immunoglobulin discovery, the first antibody immunodeficiency were recognized and named as "
agammaglobulinemia", followed by the common variable immunoendeficiency and the
hyper-IgM syndrome. The following discoveries in immunology history made possible the understanding of these pathologies, for example: the discoveries of B cells, pre-B cells, the signaling pathway directed by the
antigen receptor and many other cellular and molecular mechanisms.
Primary antibody deficiencies have been studied for a long time and the discoveries of new syndromes have been helpful in the understanding of immunological mechanisms that take place in our organism. Then, this manuscript pretends to review the relevant findings in the history of immunology, focused on the B cells and the connection with the description of representative clinical entities of
primary antibody deficiencies. The aim of this manuscript is to show to the reader that the generation of scientific knowledge has a direct application in the understanding of the molecular mechanisms that are affected in these diseases.