Bruton's agammaglobulinaemia is one of the most common hereditary immunodeficiency syndromes. The basic defect is a lack of mature B cells; there is a block in maturation after the pre-B cell stage. Lymph nodes and spleen lack germinal centres, and plasma cells are absent from all tissues. Pre-B cells may be present normally in marrow and T cell function is essentially intact.
The condition is inherited as an X linked recessive disorder (only males are affected).
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