Primary Immunodeficiency Disorders:These disorders arise from inherited defects that heighten vulnerability to infections. They differ from secondary immune deficiencies, which result from exposure to infections, chemicals, or radiation. A prime example of an acquired immune deficiency is AIDS. It is believed that there are about 100 distinct primary immunodeficiency disorders, all genetic in origin, where certain immune system cells fail to operate correctly. Symptoms vary widely, from mild or absent, as seen in selective IgA deficiency, to severe, as observed in severe combined immunodeficiency (SCID), commonly known as "bubble-boy" syndrome.While increased infection risk is a significant consequence of these conditions, they can also lead to other health issues, including allergies, joint inflammation, digestive problems, growth delays, or liver enlargement. Individuals with primary immune deficiencies often require treatments such as intravenous immunoglobulin infusions, various therapies, or even bone marrow transplants.The prevalence of primary immune deficiency disorders varies. For instance, selective IgA deficiency is relatively common, affecting 1 in 500 to 1 in 1000 people, whereas SCID is quite rare, impacting about one in a million individuals. In the United States, around 25,000 to 50,000 people suffer from severe forms of these disorders.
