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Review
. 2016 Apr;83(4):331-7.
doi: 10.1007/s12098-015-2024-8. Epub 2016 Feb 24.

X-linked Agammaglobulinemia

Deepti Suri  1 , Amit Rawat  2 , Surjit Singh  2
Affiliations
Review

X-linked Agammaglobulinemia

Deepti Suri et al. Indian J Pediatr. 2016 Apr.

Abstract

X-linked agammaglobulinemia (XLA) is one of the commonest primary immune deficiencies encountered in pediatric clinical practice. In adults, common variable immunodeficiency (CVID) is the most common primary immunodeficiency disease (PID). It is an X-linked disorder characterized by increased susceptibility to encapsulated bacteria, severe hypergammaglobulinemia and absent circulating B cells in the peripheral blood. Replacement immunoglobulin therapy is the main cornerstone of treatment. Aggressive management of intercurrent infections and prophylactic antimicrobials are needed. This review attempts to highlight varied clinical manifestations and management of XLA, especially in the context of developing country.

Keywords: Antibody deficiency; Brutons disease; Hypogammaglobulinemia; Immunoglobulins.

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