How is X-linked agammaglobulinemia (XLA) differentiated from Wiskott-Aldrich syndrome?

Updated: Apr 28, 2021
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Harumi Jyonouchi, MD  more...
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Answer

Wiskott-Aldrich syndrome is sometimes confused with Bruton agammaglobulinemia (X-linked agammaglobulinemia [XLA]) when the infant presents with recurrent otitis media, and when quantitative immunoglobulin levels show low immunoglobulin G (IgG). Patients with XLA are unlikely to have bleeding related to thrombocytopenia. Typically, Wiskott-Aldrich syndrome is associated with low immunoglobulin M (IgM) levels and normal-to-high immunoglobulin A (IgA) levels, whereas all immunoglobulin levels are undetectable in XLA. T-cell and B-cell population patterns are also characteristically different (normal CD19+ B cells and high CD4:CD8 ratios in Wiskott-Aldrich syndrome compared with absent CD19+ B cells and normal-to-elevated T cells in XLA).


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