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Find detailedTypes of PI
The World Health Organization recognizes over 150 types of primary immunodeficiencies (PIs). However, fewer than 20 of these diseases account for over 90% of cases.1
The list below represents some of the more common PIs. You will find information about clinical presentation, diagnosis, genetics, and treatment for each listed PI.
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1. B-Cell/Antibody Deficiencies (Humoral Deficiency)
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X-Linked Agammaglobulinemia (XLA)
- Conley ME. Clinical Findings Leading to the Diagnosis of X-linked Agammaglobulinemia.
Journal of Pediatrics. 2002 Oct;141(4):566-71. (View Abstract) - European Society for Immunodeficiencies
- Immune Deficiency Foundation Handbook
- Conley ME. Clinical Findings Leading to the Diagnosis of X-linked Agammaglobulinemia.
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Common Variable Immunodeficiency
- Cunningham-Rundles C. Clinical and Immunologic Studies of Common Variable Immunodeficiency. Current Opinion in Pediatrics. 1994 Dec;6(6):676-81 (View Abstract)
- Cunningham-Rundles C., Bodian C. Common variable immunodeficiency: clinical and immunological features of 248 patients. Clinical Immunology. 1999 Jul;92(1):34-48 (View Abstract)
- European Society for Immunodeficiencies
- Immune Deficiency Foundation Handbook
- National Organization for Rare Disorders
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Selective IgA Deficiency
- Cunningham-Rundles C. Physiology of IgA and IgA Deficiency. Journal of Clinical Immunology. 2001 Sep;21(5):303-9 (View Abstract)
- European Society for Immunodeficiencies
- Immune Deficiency Foundation Handbook
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IgG Subclass Deficiency
- Immune Deficiency Foundation Handbook
- Morell A. Clinical Relevance of IgG Subclass Deficiencies. Annales de Biologie Clinique (Paris). 1994;52(1):49-52 (View Abstract)
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2. Combined Deficiencies (Cellular Deficiency)
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Severe Combined Immunodeficiencies (SCID)
- Buckley RH, et al. Human severe combined immunodeficiency: genetic, phenotypic, and functional diversity in one hundred eight infants. Journal of Pediatrics. 1997 Mar;130(3):378-87 (View Abstract)
- European Society for Immunodeficiencies
- Gennery AR. Diagnosis of Severe Combined Immunodeficiency. Journal of Clinical Pathology. 2001 Mar;54(3):191-5. (View Abstract)
- Immune Deficiency Foundation Handbook
- National Organization for Rare Disorders
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Hyper IgM Syndrome
- Immune Deficiency Foundation Handbook
- Levy J. Clinical Spectrum of X-linked Hyper-IgM Syndrome. Journal of Pediatrics. 1997 Jul;131(1 Pt 1):47-54 (View Abstract)
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3. Other Well-Defined Deficiencies
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DiGeorge Syndrome
- European Society for Immunodeficiencies
- Immune Deficiency Foundation Handbook
- Markert ML. Complete DiGeorge Syndrome: Persistence of Profound Immunodeficiency. Journal of Pediatrics. 1998 Jan;132(1):15-21 (View Abstract)
- National Organization for Rare Disorders
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Hereditary Ataxia-Telangectasia
- European Society for Immunodeficiencies
- Immune Deficiency Foundation Handbook
- Perlman S. Ataxia-Telangiectasia: Diagnosis and Treatment. Seminars in Pediatric Neurology Neurol. 2003 Sep;10(3):173-82 (View Abstract)
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Wiskott-Aldrich Syndrome
- European Society for Immunodeficiencies
- Immune Deficiency Foundation Handbook
- National Organization for Rare Disorders
- Nonoyama S. Wiskott-Aldrich Syndrome. Current Allergy and Asthma Reports. 2001 Sep;1(5):430-7 (View Abstract)
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4. Phagocytic Deficiencies
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Chronic Granulomatous Disease (CGD)
- European Society for Immunodeficiencies
- Goldblatt D. Current Treatment Options for Chronic Granulomatous Disease. Expert Opinion on Pharmacotherapy. 2002 Jul;3(7):857-63 (View Abstract)
- Immune Deficiency Foundation Handbook
- National Organization for Rare Disorders
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Chediak-Higashi Syndrome
- National Organization for Rare Disorders
- Ward DM. Chediak-Higashi Syndrome: a Clinical and Molecular View of a Rare Lysosomal Storage Disorder. Current Molecular Medicine. 2002 Aug;2(5):469-77 (View Abstract)
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5. Complement Deficiencies
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- Wen L. Clinical and Laboratory Evaluation of Complement Deficiency. Journal of Allergy and Clinical Immunology. 2004 Apr;113(4):585-93 (View Abstract)
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6. Other Hereditary Immunodeficiency Diseases
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Hyper IgE Syndrome (Job Syndrome)
- Garraud O. Regulation of immunoglobulin Production in Hyper-IgE (Job's) Syndrome. Journal of Allergy and Clinical Immunology 1999; 103:333-40 (View Abstract)
- Grimbacher. Hyper-IgE Syndrome with Recurrent Infections - An Autosomal Dominant Multisystem Disorder. New England Journal of Medicine. 1999; 340: 692-702 (View Abstract)
- National Organization for Rare Disorders
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These links provide a list of web sites about immune diseases that may be of interest to healthcare professionals. Baxter Healthcare Corporation does not possess editorial control over the content or the information on those other sites. As such, Baxter Healthcare Corporation and the compiler of the list make no representation as to the accuracy, completeness and any other aspect of the information contained in those sites.
Study abstracts have been provided to assist you in selecting literature that may be of interest in your practice. As a professional courtesy, we will be happy to provide full text articles upon request. Please feel free to contact our Medical Information-Professional Services department toll-free at 1-866-4BIOSCI (424-6724).
- Applying Public Health Strategies to Primary Immunodeficiency Diseases. CDC:MMWR. January 16, 2004. 53(RR01);p.1.
