Agammaglobulinemia is a rare inherited immunodeficiency disorder, characterized by a complete absence of mature B cells, which can result in severe antibody deficiency and recurrent infections 1. This is in contrast to hypogammaglobulinaemia where there is a reduction in all types of gammaglobulins without a total absence.
It tends to manifest in infants as soon as the protective effect of maternal immunoglobulins wanes at around six months of age.
Types
Agammaglobulinemia can occur in the following types 1-4:
From an imaging perspective, it is important to recognize the effects of multiple infections within the body.
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