Immunodeficiency

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Immunodeficiency Classification and external resources
ICD-10	D84.9
ICD-9	279.3
DiseasesDB	21506
MeSH	D007153

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Overview

In medicine, immunodeficiency (or immune deficiency) is a state in which the immune system's ability to fight infectious disease is compromised or entirely absent. Most cases of immunodeficiency are acquired ("secondary") but some people are born with defects in the immune system, or primary immunodeficiency. A person who has an immunodeficiency of any kind is said to be immunocompromised. An immunocompromised person may be particularly vulnerable to opportunistic infections, in addition to normal infections that could affect everyone.

Primary immunodeficiency

Main article: Primary immunodeficiency

A number of rare diseases feature a heightened susceptibility to infections from childhood onward. Many of these disorders are hereditary and are autosomal recessive or X-linked. There are over 80 recognised primary immunodeficiency syndromes; they are generally grouped by the part of the immune system that is malfunctioning, such as lymphocytes or granulocytes.^[1]

The treatment of primary immunodeficiencies depends on the nature of the defect, and may involve antibody infusions, long-term antibiotics and (in certain cases) stem cell transplantation.

Acquired immunodeficiency

Immune deficiency may also be the result of particular external processes or diseases; the resultant state is called "secondary" or "acquired" immunodeficiency. Common causes for secondary immunodeficiency are malnutrition, aging and particular medications (e.g. chemotherapy, disease-modifying antirheumatic drugs, immunosuppressive drugs after organ transplants, glucocorticoids).

Many specific diseases directly or indirectly impair the immune system. This include many types of cancer, particularly those of the bone marrow and blood cells (leukemia, lymphoma, multiple myeloma), and certain chronic infections. Immunodeficiency is also the hallmark of acquired immunodeficiency syndrome (AIDS), caused by the human immunodeficiency virus (HIV). HIV directly attacks the immune system.

References

1. ↑ Rosen FS, Cooper MD, Wedgwood RJ (1995). "The primary immunodeficiencies". N. Engl. J. Med. 333 (7): 431–40. PMID 7616993.

v • d • e Immune system / Immunology
Systems	Adaptive immune system vs. Innate immune system • Humoral immune system vs. Cellular immune system • Complement system (Anaphylatoxins) • Intrinsic immune system
Antibodies and antigens	Antibody (Monoclonal antibodies, Polyclonal antibodies, Autoantibody) • Allotype • Isotype • Idiotype • Antigen (Superantigen)
Immune cells	White blood cells (T cell, B cell, NK cell, Mast cell, Basophil, Eosinophil) • Phagocyte (Neutrophil, Macrophage, Dendritic cell) • Antigen-presenting cell • Reticuloendothelial system
Immunity vs. tolerance	Immunity • Autoimmunity • Allergy • Tolerance (Central) • Immunodeficiency
Immunogenetics	Somatic hypermutation • V(D)J recombination • Immunoglobulin class switching • MHC / HLA
Substances	Cytokines • Opsonin • Cytolysin
Other	Inflammation • Epitope (Hapten) • Cross-reactivity

v • d • e Pathology: Immune disorders (primarily D80-D89, 273, 279)
Immunodeficiency/ primary	Antibody/humoral (B) Hypogammaglobulinemia/agammaglobulinemia (X-linked, Transient of infancy) - Dysgammaglobulinemia (IgA, IgG) - Common variable immunodefiency - Leukocyte adhesion deficiency - WHIM syndrome Cell-mediated (T) DiGeorge syndrome - Nezelof syndrome Combined (B+T) Severe combined immunodeficiency (Adenosine deaminase deficiency, Omenn syndrome, X-SCID, ZAP70 deficiency) - Purine nucleoside phosphorylase deficiency - Bare lymphocyte syndrome - Wiskott-Aldrich syndrome Complement deficiency Angioedema PBD Chédiak-Higashi syndrome - Chronic granulomatous disease - Hyper-IgE syndrome Other ICF syndrome
Immunoproliferative	Hypergammaglobulinemia - Paraproteinemia (Cryoglobulinemia, Heavy chain disease, POEMS syndrome, Monoclonal gammopathy of undetermined significance)
Autoimmunity	see list of autoimmune diseases
Other	Sarcoidosis - Hypersensitivity - Myeloperoxidase deficiency
See also hematological malignancy and hematology

ca:Immunodeficiència

de:Immundefekt el:Ανοσολογική ανεπάρκειαfr:Immunodépression he:כשל חיסוני

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Acknowledgement and Attribution Regarding Sources of Content

Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .