Lior Keren David MD, Ilan Dalal MD, Adi Ovadia MD
Agammaglobulinemia was the first primary immunodeficiency identified in humans. It is characterized by an absent or significantly reduced number of mature B cells, decreased serum immunoglobulins, and recurrent infections beginning in infancy. The entity was first described in 1952 by Colonel Ogden Bruton at the Walter Reed Army Hospital in the United States. Bruton reported on an 8-year-old boy who had experienced 19 episodes of pneumonia over 4 years and was found to have a complete absence of antibody-containing γ-globulins in his serum [1].
Over time, through improvement in the immune and genetic investigations, our understanding of agammaglobulinemia has expanded enabling earlier diagnosis and treatment resulting in a change of the disease course.