In which sex-linked immunodeficiency disorder is there an absence of plasma cells resulting in the lack of antibody production?

Bruton's agammaglobulinemia, also known as X-linked agammaglobulinemia, is characterized by a significant absence of plasma cells, leading to severely reduced or absent antibodies in affected individuals. This disorder is caused by mutations in the BTK gene, which plays a vital role in B-cell development. As a result, patients with Bruton's agammaglobulinemia have a marked deficiency in mature B cells, which are responsible for producing antibodies.

Without adequate B cells and their subsequent maturation into plasma cells, individuals with this immunodeficiency are highly susceptible to recurrent bacterial infections due to their inability to mount an effective humoral immune response. In contrast, other disorders mentioned involve different mechanisms or do not specifically result in a lack of plasma cells leading to insufficient antibody production. For instance, common variable immunodeficiency involves impaired antibody production as well, but typically has functioning plasma cells. Hyper-IgM syndrome is characterized by the presence of IgM antibodies due to a specific defect, and chronic granulomatous disease affects the ability of phagocytes to kill certain bacteria but does not directly concern antibody production.