Primary Immune Deficiency

The information provided is not intended to be a substitute for professional medical advice. A licensed healthcare professional should be consulted for diagnosis and treatment of any and all medical conditions.

About Primary immune deficiency

Primary immune deficiency (PI) is actually the name for a group of over 150 diseases1 that:

  • Are usually inherited
  • Are caused by errors in the genes of the cells that make up the immune system
  • Have a wide range of symptoms ranging from mild to severe

 

People with primary immune deficiency diseases have immune systems that lack one or more types of immune tissues, proteins, or cells.

The immune system protects the body from germs like bacteria and viruses. So, if part of your immune system is missing or not functioning correctly, you'll be more likely to get infections, you may take longer to recover, even with antibiotic treatment, and you may have recurring infections.

Susceptibility to infection is one of the most common symptoms of primary immune deficiency. It can often be seen early in a child's life. However, signs of immune deficiency may also occur in older children, teenagers, or adults.

Types of PI

Primary immunodeficiency (PI) diseases differ in many ways. Some types of PI are characterized by low levels of antibodies. Others involve defects in T cells, B cells, granulocytes or the complement system. Because of these differences, individuals with different types of PI are susceptible to different types of diseases.

Currently the World Health Organization lists over 150 different types of primary immunodeficiency. 1 Different therapies are available for different types of PI. And healthy living habits can improve outcomes as well.

The most common types of PI include

  • X-Linked Agammaglobulinemia (XLA)
  • Common Variable Immunodeficiency (CVID)/ Hypogammaglobulinemia
  • Hyper-IgM Syndrome
  • Selective IgA Deficiency
  • IgG Subclass Deficiency
  • Severe Combined Immunodeficiency (SCID)
  • Wiskott-Aldrich Syndrome
  • DiGeorge Syndrome
  • Ataxia-telangiectasia

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Intravenous immunoglobulin (IVIG) therapy2

There are a number of specific medical therapies that can help people diagnosed with primary immunodeficiencies (PI) lead healthier lives. One of the most common therapies is intravenous immunoglobulin, or IVIG.

Intravenous immunoglobulin (IVIG) therapy is helpful to some people with primary immunodeficiency (PI) because it temporarily replaces infection-fighting antibodies that some PI patients are missing.

This purified plasma treatment contains antibodies collected from the plasma of healthy donors and purified through a special process so that the therapy is safe and effective. Most of the antibodies are of the IgG class of antibodies, also called immunoglobulin G or gamma globulin.

IVIG is given intravenously, which means through a needle directly into a vein. Your doctor will determine the correct dosage, based on a number of individual factors, including your weight, your condition, and how well the IVIG treats or prevents symptoms.

IVIG can be administered in a hospital outpatient unit, infusion center, or your home. A typical infusion takes 2-4 hours, although some patients may require slower infusions to avoid side effects. 

IVIG replaces antibodies the body should be making, but does not help the patient's own immune system make more, so repeat doses are needed, usually every 3 to 4 weeks.

Since primary immunodeficiency patients aren't "cured" by IVIG, they can still contract an infection, but infections have been shown to be less frequent and less severe.

PI patients who rely on IVIG generally need infusions throughout their lives.

References

1. Geha R, Notarangelo L, Casanova JL, Conley ME, Chapel ME, Fischer A, Hammerstrom L, Nonoyama S, Ochs H, Puck J, Roifman C, Seger R, Wedgwood J. Primary immunodeficiency diseases: an update from the International Union of Immunological Societies Primary Immunodeficiency Diseases Classification Committee Meeting. Journal of Allergy and Clinical Immunology 2007; 120(4):776-794.

2. Patient & Family Handbook for Primary Immunedeficiency Diseases Produced by Immune Deficiency Foundation through a grant from Baxter. Fourth Edition. 2007.

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