For some patients for whom the exact diagnosis is still unclear, it may be important to test the function of these immunoglobulins that are present. These patients are unable to respond with antibody formation following immunization. When a specific defect is found in the affected male, research laboratories can then test female members to diagnose those who carry the abnormal X chromosomes. White blood cell counts are normal, but B-lymphocytes, measured by immunofluorescent techniques in the laboratory are usually absent. There is no cure for X-Linked Agammaglobulinemia at this time.
Treatment focuses on providing antibody replacement through gammaglobulin therapy.
This treatment helps these patients by providing them with the antibodies they cannot make to protect against the spread of infections into the bloodstream and to body organs and tissues. In patients who have developed recurrent or chronic infections, it may be necessary to obtain specimens of sputum, stool, or the infected tissue in order to allow institution of specific antibiotic therapy. These patients should not be given live viral vaccines because they could develop the disease for which the vaccination was given. Polio is an example. Prognosis Without gammaglobulin treatment, these patients may die from infections at an early age.