Definition for x linked agammaglobulinemia

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. more

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. XLA patients do not generate mature B cells capable of manufacturing antibodies called immunoglobulins that the body uses to defend itself from infection. 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. less