Indication

Agammaglobulinemia

Agammaglobulinemia is a rare primary humoral immunodeficiency disorder characterized by an early developmental arrest in B-cell maturation within the bone marrow. Read more about Agammaglobulinemia

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About Agammaglobulinemia.

Agammaglobulinemia is a rare primary humoral immunodeficiency disorder characterized by an early developmental arrest in B-cell maturation within the bone marrow.

This disruption leads to an absence or profound deficiency of mature circulating peripheral B cells and a marked reduction across all major serum immunoglobulin isotypes.

Primarily manifesting in infancy or early childhood following the decay of maternal antibodies, the condition leaves patients vulnerable to recurrent, severe sinopulmonary and systemic infections caused predominantly by encapsulated bacteria and certain enteroviruses.

The disease occurs most frequently as an X-linked recessive trait, although various autosomal recessive and dominant forms have also been identified.

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