Primary immune deficiency diseases are inherited disorders in which part of the body's immune system is missing or does not function properly. There are a wide variety of primary immune deficiencies.

The World Health Organisation recognises approximately 70 primary immune deficiencies including X-Linked Agammaglobulinemia (Bruton's Disease), Common Variable Immune Deficiency (also called Hypogammaglobulinemia), Selective IgA Deficiency, and Severe Combined Immune Deficiency (boy-in-the-bubble disease).

Some disorders such as Selective IgA Deficiency can be quite common, occurring as often as 1/500 to 1/1000 individuals. Others may be as rare as 1 individual affected per million. Untreated primary immune deficiencies are characterised by frequent life threatening infections and debilitating illnesses.

Because of advances in our medical understanding and treatment of primary immune deficiency diseases, many patients who in the past would not have survived childhood are now able to live nearly normal lives. Most primary immune deficient patients require life long therapies including intravenous gammaglobulin infusion, aggressive antibiotic therapies, or bone marrow transplantation.

For further information try the below links