X-linked agammaglobulinemia (XLA)

The Primary Immunodeficiency Program at Children’s Health℠ has been recognized as a Center of Excellence by the Jeffrey Modell Foundation for the diagnosis and treatment of patients with primary immunodeficiency including XLA.

What is X-linked agammaglobulinemia (XLA)?

X-linked agammaglobulinemia (XLA) is an inherited (congenital) immunodeficiency disease in which the body is unable to produce antibodies needed to defend against pathogens (bacteria, viruses, fungi).

How is X-linked agammaglobulinemia (XLA) treated?

If your child is diagnosed with XLA, they will be treated with immunoglobulin IgG (antibodies) therapy on a regular basis for the rest of his life. IgG therapy can be given through a catheter in your child’s vein (intravenous; IVIG) or subcutaneously (SCIG). The decision to use one or the other depends on what’s best for your child and your family. There is currently no cure for XLA.

Children with XLA should be followed at the center. If your child develops an infection, antibiotics can treat the infection. Some patients with XLA may have complications including gastrointestinal problems that may require cooperative interaction with gastroenterology specialists.

X-linked agammaglobulinemia (XLA) doctors and providers

Frequently Asked Questions

  • What is the prognosis for children diagnosed with X-linked agammaglobulinemia (XLA)?

    Most individuals with XLA who receive immunoglobulin on a regular basis can lead normal, healthy lives.

  • Should my other children be tested for X-linked agammaglobulinemia (XLA))?

    If a newborn baby has a brother, sister, maternal cousin or maternal uncle with agammaglobulinemia, the baby is at risk and should immediately be evaluated by an immunologist who will determine what tests will need to be done.

    These usually include antibody levels in the blood, determination of circulating numbers of B-cells in the blood, and, if a known genetic diagnosis is available, confirmatory testing for both affected and carrier status can be performed.