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Agammaglobulinemia   

Overview | Symptoms | Treatment | Prevention

Alternative names:

Bruton's agammaglobulinemia; X-linked agammaglobulinemia

Definition:

An inherited disorder characterized by very low levels of protective immunoglobulins; affected people develop repeated infections.

Causes and Risks

Agammaglobulinemia is rare disorder that only affects male as it is inherited as an X-linked recessive trait (like hemophilia). It is the result of a genetic abnormality that blocks normal B-lymphocyte differentiation (development into nromal mature cell). As a consequence, the body produces 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 bacterial infections caused by Hemophilus influenzae, pneumococcus (Streptococcus pneumoniae), and staphylococci as well as repeated viral infections. The upper respiratory tract, lungs, and skin are common sites of infection.

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