This is an inherited disorder characterized by very low levels of protective immunoglobulins. People suffering from this condition develop repeated infections. Agammaglobulinemia is a rare disorder that mainly affects males.

Agammaglobulinemia is inherited as an X-linked recessive trait in which an inborn error blocks B-cell differentiation, causing the body to produce very little (if any) gamma globulin (IgG) and other immunoglobulins in the bloodstream. Immunoglobulins are molecules that play a significant role in the immune response, which protects against illness and infection. In the absence of protective immunoglobulins, affected individuals develop repeated infections. People with this disorder are particularly susceptible to Hemophilus influenza, pneumococcus infections, staphylococcus infections and repeated viral infections. The upper respiratory tract, lungs, and skin are common sites of infection.

Genetic counseling may be provided to prospective parents with a family history of agammaglobulinemia or other immunodeficiency disorders, thus preparing them for intervention at an early stage.