Jerry A. Winkelstein

All articles by Jerry A. Winkelstein

X-linked Agammaglobulinemia (Congenital Agammaglobulinemia, Bruton's Agammaglobulinemia

Are You Confident of the Diagnosis? X-linked agammaglobulinemia (XLA) is a primary immunodeficiency characterized by an intrinsic defect in the maturation of pre-B-cells to B-cells and ultimately immunoglobulin-secreting plasma cells. As a result, affected patients (male) have markedly reduced serum immunoglobulin levels and nearly absent antibody levels. Since the disorder is the result of mutations…

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