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What is a Primary
Immune Deficiency Disease?
from the Michigan Immunodeficiency Foundation
https://www.bridges4kids.org/Disabilities/ImmuneDeficiency.html
Primary immune deficiency diseases are disorders in which part of
the body's immune system is missing or does not function properly. In
contrast to secondary immune deficiency disease in which the immune
system is compromised by factors outside the immune system, such as
with certain medications like chemotherapy or viruses, the primary
immune deficiency diseases are caused by genetic or intrinsic defects
in the immune system.
There are a wide variety of primary immune deficiencies. There are
nearly 100 primary immune deficiency diseases 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 1/400 individuals. While others,
such as Severe Combined Immune Deficiency, can be as rare as one in a
million. Untreated primary immune deficiencies may be characterized by
frequent life-threatening or chronic infections and debilitating
illnesses.
With advances in medicine and our increasing knowledge of primary
immune deficiency diseases, treatment of primary immune deficient
patients who in the past may not have even survived childhood are now
capable of living nearly normal lives. Many individuals affected by
primary immune deficiency diseases require life long therapies
including intravenous gamma globulin infusions (IVIG), antibiotic
therapies, or bone marrow transplantation. The Michigan
Immunodeficiency Foundation is dedicated to improving the diagnosis
and treatment of primary immune deficiency diseases through research
and education.
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