The diagnosis of XLA was based on the recommendation of WHO: Severe reduction in all serum immunoglobulin iso-type, profound deficiency or absence of B cell, X linked inheritance, mutations in Burton tyrosine kinases and severe bacterial infections. Results: A total of 4 male cases were found to have defined XLA with the following immunologic characteristics: Low isotype immunoglobulinemia median with IgG (25.5mg / dL), IgM (13.5mg / dL), IgA (10mg / dL), and decreased circulating B cell level (6%).