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X-linked and Autosomal Recessive Agammaglobulinemia (Pediatric)

What is X-linked and autosomal recessive agammaglobulinemia?

X-Linked Agammaglobulinemia (XLA; Bruton’s agammaglobulinemia) and Autosomal Recessive Agammaglobulinemia (ARA) occur due to failure of specific immune cells called B-lymphocyte precursors to mature into B cells and then the plasma cells needed to produce immunoglobulins.

What are the symptoms of X-linked and autosomal recessive agammaglobulinemia?

This disorder great increases the likelihood of infections, particularly of the middle ear (otitis), sinuses and lungs, as well as serious ones involving the bloodstream or internal organs.

How is X-linked and autosomal recessive agammaglobulinemia treated?

A specialized immunologist is recommended to evaluate any child that displays some of these characteristics. The immunologist likely would screen by examining whether the patient’s tonsils and lymph nodes are small or absent.

A screening blood test would be measurement of immunoglobulins to determine whether they are markedly reduced or absent. Additional testing involves measuring the number of B-cells in the peripheral blood and genetic testing.

Management may include immunoglobulin replacement, frequent antibiotics to treat infections, and avoidance of live vaccines.