ImmunoDeficiency Resource (IDR)

Most patients with XLA develop recurrent bacterial infections, particularly otitis, sinusitis and pneumonia, in the first two years of life. The most common organisms are S. pneumonea and H. influenzae. The serum IgG is usually less than 200 mg/dl and the IgG and IgA are generally less than 20 mg/dl. Approximately 20% of patients present with a dramatic, overwhelming infection, often with neutropenia. Another 10-15% have higher concentrations of serum immunoglobulin than expected or are not recognized to have immunodeficiency until after 5 years of age. Complications can lead to bronchiectasis, chronic sinus damage, and also chronic meningoencephalitis due to echoviruses and coxsackieviruses. Ureaplasma/Mycoplasma septic arthritis may occur.