Primary immunodeficiency

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Causes

By Mayo Clinic staff

Many primary immunodeficiency disorders are inherited — passed down from one or both parents. Problems in the DNA — the genetic code that acts as a blueprint for producing the cells that make up the human body — cause many of the immune system defects in primary immunodeficiency.

There are numerous types of primary immunodeficiency disorders. They can be broadly classified into six groups based on the part of the immune system that's affected:

  • B cell (antibody) deficiencies
  • T cell deficiencies
  • Combination B and T cell deficiencies
  • Defective phagocytes
  • Complement deficiencies
  • Unknown (idiopathic)

B cell deficiencies are the most common type of primary immunodeficiency disorder.

References
  1. Primary immunodeficiency. Eunice Kennedy Shriver National Institute of Child Health and Human Development. http://www.nichd.nih.gov/publications/pubs/primary_immuno.cfm. Accessed June 12, 2011.
  2. Specific medical therapy. In: Blaese R, et al. IDF Patient and Family Handbook for Primary Immunodeficiency Diseases. 4th ed. Towson, Md.: Immune Deficiency Foundation; 2007. http://www.primaryimmune.org/publications/book_pats/patient_and_family_handbook_4th.pdf. Accessed June 12, 2011.
  3. Primary immunodeficiency diseases. American Academy of Allergy, Asthma and Immunology. http://www.aaaai.org/patients/topicofthemonth/0407/. Accessed June 12, 2011.
  4. Immunodeficiency disorders. The Merck Manuals: The Merck Manual for Healthcare Professionals. http://www.merckmanuals.com/professional/print/sec13/ch164/ch164a.html. Accessed June 12, 2011.
  5. Fischer A, et al. Gene therapy for primary adaptive immune deficiencies. Journal of Allergy and Clinical Immunology. 2011;127:1356.
DS01006 July 28, 2011

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