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

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. Transplant patients take medications to suppress their immune system as an anti-rejection measure, as do some patients suffering from an over-active immune system. 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.

Contents 1 Types 1.1 Primary immunodeficiency 1.2 Acquired immunodeficiency 2 References 3 External links

[edit] Types

[edit] 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 some cases) stem cell transplantation.

[edit] 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 includes 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 infects a small number of T helper cells, and also impairs other immune system responses indirectly.

[edit] References

1. ^ Rosen FS, Cooper MD, Wedgwood RJ (1995). "The primary immunodeficiencies". N. Engl. J. Med. 333 (7): 431–40. doi:10.1056/NEJM199508173330707. PMID 7616993. 

[edit] External links

• Immune Deficiency Foundation

v • d • e Pathology: Medical conditions (Diseases/Disorders/Illness, Syndromes/Sequences, Symptoms/Signs, Injuries, etc.) and ICD codes (A/B, 001-139) Infectious disease/Infection: Bacterial disease (G+, G-) · Virus disease · Parasitic disease (Protozoan infection, Helminthiasis, Ectoparasitic infestation) · Mycosis · Zoonosis (C/D, 140-239 & 280-289) Cancer (C00-D48, 140-239) Tumor Myeloid hematologic (D50-D77, 280-289) Anemia · Coagulopathy Lymphoid immune (D80-D89, 279) Immunodeficiency · Immunoproliferative disorder · Hypersensitivity (E, 240-278) Endocrine disease · Nutrition disorder · Inborn error of metabolism (F, 290-319) Mental disorder (G, 320-359) Nervous system disease (CNS, PNS) · Neuromuscular disease (H, 360-389) Eye disease · Ear disease (I, 390-459) Cardiovascular disease (Heart disease, Vascular disease) (J, 460-519) Respiratory disease (Obstructive lung disease, Restrictive lung disease, Pneumonia) (K, 520-579) Stomatognathic disease (Tooth disease) · Digestive disease (Esophageal, Stomach, Enteropathy, Liver, Pancreatic) (L, 680-709) Skin disease · skin appendages (Nail disease, Hair disease, Sweat gland disease) (M, 710-739) Musculoskeletal disorders · Osteochondropathy (Osteopathy, Chondropathy) (N, 580-629) Urologic disease (Nephropathy, Urinary bladder disease) · Male genital disease · Breast disease · Female genital disease (O, 630-679) Complications of pregnancy · Obstetric labor complication · Puerperal disorder (P, 760-779) Fetal disease (Q, 740-759) Congenital disorder (R, 780-799) Syndromes · Medical signs (Eponymous) (S/T, 800-999) Bone fracture · Joint dislocation · Sprain · Strain · subluxation · Head injury · Chest trauma · Poisoning

v • d • e Immune system / Immunology Systems Adaptive vs. Innate · Humoral vs. Cellular · Complement (Anaphylatoxins) · Intrinsic Lymphoid Antigens Antigen (Superantigen, Allergen) · Hapten Epitope (Linear, Conformational) Antibodies Antibody (Monoclonal antibodies, Polyclonal antibodies, Autoantibody) · Polyclonal B cell response · Allotype · Isotype · Idiotype Immune complex Immunity vs. tolerance action: Immunity · Autoimmunity · Alloimmunity · Allergy · Inflammation · Cross-reactivity inaction: Tolerance (Central, Peripheral, Clonal anergy, Clonal deletion, Tolerance in pregnancy) · Immunodeficiency Immunogenetics Somatic hypermutation · V(D)J recombination · Junctional diversity  · Immunoglobulin class switching · MHC/HLA Immune cells/ White blood cells Lymphoid: T cell · B cell · NK cell Myeloid: Mast cell · Basophil · Eosinophil · Phagocytes (Neutrophil, Macrophage/Reticuloendothelial system) Professional APCs: Dendritic cell · Macrophage · B cell Substances Cytokines · Opsonin · Cytolysin Lymphopoiesis Lymphoblast · Prolymphocyte Other Diagnostic immunology · Plant disease resistance lymphocyte navs: cells/physio, immunodeficiency/immunoproliferative immunoglobulin/neoplasia, proc

v • d • e Immune disorders: Lymphoid and complement immunodeficiency (D80-D85, 279) Primary Antibody/humoral (B) Hypogammaglobulinemia X-linked agammaglobulinemia · Transient hypogammaglobulinemia of infancy Dysgammaglobulinemia IgA deficiency · IgG deficiency · IgM deficiency · Hyper IgM syndrome (2, 3, 4, 5) · Wiskott-Aldrich syndrome · Hyper-IgE syndrome Other Common variable immunodeficiency · ICF syndrome Cell-mediated (T) thymic hypoplasia: hypoparathyroid (Di George's syndrome) · euparathyroid (Nezelof syndrome, Ataxia telangiectasia) peripheral: Purine nucleoside phosphorylase deficiency · Hyper IgM syndrome (1) Severe combined (B+T) x-linked: X-SCID autosomal: Adenosine deaminase deficiency · Omenn syndrome · ZAP70 deficiency · Bare lymphocyte syndrome Acquired AIDS Lymphocytopenia Idiopathic CD4+ lymphocytopenia Complement deficiency C1-inhibitor (Angioedema/Hereditary angioedema) · Complement 2 deficiency lymphocyte navs: cells/physio, immunodeficiency/immunoproliferative immunoglobulin/neoplasia, proc

v • d • e Hematologic disease: Monocyte and granulocyte disease (CFU-GM/CFU-Baso/CFU-Eos), including immunodeficiency (D70-D71, 288) Monocytes/ macrophages ↑ Histiocytosis · Chronic granulomatous disease -cytosis: Monocytosis ↓ -penia: Monocytopenia Granulocytes ↑ -cytosis: granulocytosis (Neutrophilia, Eosinophilia/Hypereosinophilic syndrome, Basophilia, Bandemia) ↓ -penia: Granulocytopenia/agranulocytosis (Neutropenia/Kostmann syndrome · Eosinopenia · Basopenia) PBD chemotaxis/degranulation (Leukocyte adhesion deficiency, Chediak–Higashi syndrome) respiratory burst (Chronic granulomatous disease, Myeloperoxidase deficiency) myeloid navs: cells/physio, disease of RBCs+megakaryocytes/monocytes+granulocytes/neoplasia, symptoms+signs/eponymous