X-linked agammaglobulinemia (also called X-linked hypogammaglobulinemia, XLA, Bruton type agammaglobulinemia) is a rare
X-linked genetic disorder that affects the body's ability to fight infection (origin of the name: A=no, gammaglobulin=Antibody). XLA patients do not generate mature
B cells. B cells are part of the immune system and normally manufacture antibodies (also called
immunoglobulins) which defends the body from infections (the
humoral response). Patients with untreated XLA are prone to develop serious and even fatal infections. Patients typically present in early childhood with recurrent
infections, particularly with extracellular, encapsulated
bacteria. XLA is an
X-linked disorder, and therefore is almost always limited to males. It occurs in a frequency of about 1 in 100,000 male newborns, and has no ethnic
predisposition. XLA is treated by infusion of human antibody. Treatment with pooled gamma globulin cannot restore a functional population of
B cells, but it is sufficient to reduce the severity and number of infections due to the
passive immunity granted by the exogenous antibodies.
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