WordNet

incapable of developing an immune response following exposure to an antigen; "immunodeficient rodents"
immunological disorder in which some part of the bodys immune system is inadequate and resistance to infectious diseases is reduced

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/05/08 13:28:43」(JST)

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Immunodeficiency (or immune deficiency) is a state in which the immune system's ability to fight infectious disease is compromised or entirely absent. Immunodeficiency may also decrease cancer immunosurveillance. Most cases of immunodeficiency are acquired ("secondary") but some people are born with defects in their 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.

Types

By affected component

Humoral immune deficiency, with signs or symptoms depending on the cause, but generally include signs of hypogammaglobulinemia (decrease of one or more types of antibodies) with presentations including repeated mild respiratory infections, and/or agammaglobulinemia (lack of all or most antibody production) which results in frequent severe infections and is often fatal.^[1]
T cell deficiency, often causes secondary disorders such as acquired immune deficiency syndrome.^[2]
Granulocyte deficiency, including decreased numbers of granulocytes (called as granulocytopenia or, if absent, agranulocytosis) such as of neutrophil granulocytes (termed neutropenia). Granulocyte deficiencies also include decreased function of individual granulocytes, such as in chronic granulomatous disease.
Asplenia, where there is no function of the spleen
Complement deficiency is where the function of the complement system is deficient

In reality, immunodeficiency often affects multiple components, with notable examples including severe combined immunodeficiency (which is primary) and acquired immune deficiency syndrome (which is secondary).

Comparison of immunodeficiencies by affected component
	Affected components	Main causes^[3]	Main pathogens of resultant infections^[3]
Humoral immune deficiency	B cells, plasma cells or antibodies	Primary humoral Multiple myeloma Chronic lymphoid leukemia AIDS	Streptococcus pneumoniae Hemophilus influenzae Pneumocystis jirovecii Giardia intestinalis Cryptosporidium parvum
T cell deficiency	T cells	Marrow and other transplantation AIDS Cancer chemotherapy Lymphoma Glucocorticoid therapy	Intracellular pathogens, including Herpes simplex virus, Mycobacterium, Listeria,^[4] and intracellular fungal infections.^[3]
Neutropenia	Neutrophil granulocytes	Chemotherapy Bone marrow transplantation Dysfunction, such as chronic granulomatous disease	Enterobacteriaceae Oral Streptococci Pseudomonas aeruginosa Enterococcus species Candida species Aspergillus species
Asplenia	Spleen	Splenectomy Trauma Sickle-cell anemia	Polysaccharide encapsulated bacteria,^[5] particularly: Streptococcus pneumoniae^[5] Haemophilus influenzae^[5] Neisseria meningitidis^[5] Plasmodium species Babesia species
Complement deficiency	Complement system	Congenital deficiencies	Neisseria species Streptococcus pneumoniae

Primary or secondary

Distinction between primary versus secondary immunodeficiencies are based on, respectively, whether the cause originates in the immune system itself or is, in turn, due to insufficiency of a supporting component of it or an external decreasing factor of it.

Primary immunodeficiency

A number of rare diseases feature a heightened susceptibility to infections from childhood onward. Primary Immunodeficiency is also known as congenital immunodeficiencies.^[6] 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.^[7]

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.

Secondary immunodeficiencies

Secondary immunodeficiencies, also known as acquired immunodeficiencies, can result from various immunosuppressive agents, for example, malnutrition, aging and particular medications (e.g. chemotherapy, disease-modifying antirheumatic drugs, immunosuppressive drugs after organ transplants, glucocorticoids). For medications, the term immunosuppression generally refers to both beneficial and potential adverse effects of decreasing the function of the immune system, while the term immunodeficiency generally refers solely to the adverse effect of increased risk for infection.

Many specific diseases directly or indirectly cause immunosuppression. 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),^[6] 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.

Immunodeficiency and autoimmunity

There are a large number of immunodeficiency syndromes that present clinical and laboratory characteristics of autoimmunity. The decreased ability of the immune system to clear infections in these patients may be responsible for causing autoimmunity through perpetual immune system activation.^[8]

One example is common variable immunodeficiency (CVID) where multiple autoimmune diseases are seen, e.g. inflammatory bowel disease, autoimmune thrombocytopenia and autoimmune thyroid disease. Familial hemophagocytic lymphohistiocytosis, an autosomal recessive primary immunodeficiency, is another example. Pancytopenia, rashes, lymphadenopathy and hepatosplenomegaly are commonly seen in these patients. Presence of multiple uncleared viral infections due to lack of perforin are thought to be responsible. In addition to chronic and/or recurrent infections many autoimmune diseases including arthritis, autoimmune hemolytic anemia, scleroderma and type 1 diabetes are also seen in X-linked agammaglobulinemia (XLA). Recurrent bacterial and fungal infections and chronic inflammation of the gut and lungs are seen in chronic granulomatous disease (CGD) as well. CGD is caused by a decreased production of nicotinamide adenine dinucleotide phosphate (NADPH) oxidase by neutrophils. Hypomorphic RAG mutations are seen in patients with midline granulomatous disease; an autoimmune disorder that is commonly seen in patients with granulomatosis with polyangiitis (Wegenr’s disease) and NK/T cell lymphomas. Wiskott-Aldrich syndrome (WAS) patients also present with eczema, autoimmune manifestations, recurrent bacterial infections and lymphoma. In autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) also autoimmunity and infections coexist: organ-specific autoimmune manifestations (e.g. hypoparathyroidism and adrenocortical failure) and chronic mucocutaneous candidiasis. Finally, IgA deficiency is also sometimes associated with the development of autoimmune and atopic phenomena.

Management

Prevention of Pneumocystis pneumonia using trimethoprim/sulfamethoxazole is useful in those who are immunocompromised.^[9]

References

^ Immunodeficiency by Dr. Saul Greenberg. University of Toronto. Last updated, on February 5, 2009
^ Medscape > T-cell Disorders. Author: Robert A Schwartz, MD, MPH; Chief Editor: Harumi Jyonouchi, MD. Updated: May 16, 2011
^ ^a ^b ^c If not otherwise specified in boxes, then reference for entries is: Page 432, Chapter 22, Table 22.1 in: Jones, Jane; Bannister, Barbara A.; Gillespie, Stephen H. (2006). Infection: Microbiology and Management. Wiley-Blackwell. ISBN 1-4051-2665-5.
^ Page 435 in: Jones, Jane; Bannister, Barbara A.; Gillespie, Stephen H. (2006). Infection: Microbiology and Management. Wiley-Blackwell. ISBN 1-4051-2665-5.
^ ^a ^b ^c ^d Brigden, M. L. (2001). "Detection, education and management of the asplenic or hyposplenic patient". American family physician 63 (3): 499–506, 508. PMID 11272299. edit
^ ^a ^b Basic Immunology: Functions and Disorders of the Immune System, 3rd Ed. 2011.
^ Rosen FS, Cooper MD, Wedgwood RJ (1995). "The primary immunodeficiencies". N. Engl. J. Med. 333 (7): 431–40. doi:10.1056/NEJM199508173330707. PMID 7616993.
^ Grammatikos A, Tsokos G (2012). "Immunodeficiency and autoimmunity: lessons from systemic lupus erythematosus". Trends Mol Med 18 (2): 101–108. doi:10.1016/j.molmed.2011.10.005. PMC 3278563. PMID 22177735.
^ Stern, A; Green, H; Paul, M; Vidal, L; Leibovici, L (Oct 1, 2014). "Prophylaxis for Pneumocystis pneumonia (PCP) in non-HIV immunocompromised patients.". The Cochrane database of systematic reviews 10: CD005590. doi:10.1002/14651858.CD005590.pub3. PMID 25269391.

External links

Immune Deficiency Foundation
The European Society of Immunodeficiencies Biennial Meeting
The European Society of Immunodeficiencies

UpToDate Contents

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1. 原発性の体液性免疫不全：概要 primary humoral immunodeficiencies an overview
2. 感染症を繰り返す小児に対するアプローチ approach to the child with recurrent infections
3. 感染症を繰り返す成人に対するアプローチ approach to the adult with recurrent infections
4. 原発性免疫不全症：マネージメントの概要 primary immunodeficiency overview of management
5. 重度複合型免疫不全（SCID）：概要 severe combined immunodeficiency scid an overview

English Journal

Patient-derived xenografts of gastrointestinal cancers are susceptible to rapid and delayed B-lymphoproliferation.

Dieter SM1,2, Giessler KM1, Kriegsmann M3, Dubash TD1, Möhrmann L1, Schulz ER1, Siegl C1, Weber S1, Strakerjahn H1, Oberlack A1, Heger U1,4, Gao J1, Hartinger EM1, Oppel F1, Hoffmann CM1, Ha N5, Brors B5, Lasitschka F3, Ulrich A4, Strobel O4, Schmidt M1,2, von Kalle C1,2,6, Schneider M4, Weichert W3,7,8, Ehrenberg KR1,9,10, Glimm H1,2, Ball CR1.
International journal of cancer.Int J Cancer.2017 Mar 15;140(6):1356-1363. doi: 10.1002/ijc.30561.
Patient-derived cancer xenografts (PDX) are widely used to identify and evaluate novel therapeutic targets, and to test therapeutic approaches in preclinical mouse avatar trials. Despite their widespread use, potential caveats of PDX models remain considerably underappreciated. Here, we demonstrate
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Low p16INK4a Expression in Early Passage Human Prostate Basal Epithelial Cells Enables Immortalization by Telomerase Expression Alone.

Graham MK1, Principessa L2, Antony L2, Meeker AK3, Isaacs JT2.
The Prostate.Prostate.2017 Mar;77(4):374-384. doi: 10.1002/pros.23276. Epub 2016 Nov 8.
BACKGROUND: There are two principal senescence barriers that must be overcome to successfully immortalize primary human epithelial cells in culture, stress-induced senescence, and replicative senescence. The p16INK4a /retinoblastoma protein (p16/Rb) pathway mediates stress-induced senescence, and is
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Adult-Derived Human Liver Stem/Progenitor Cells Infused 3 Days Postsurgery Improve Liver Regeneration in a Mouse Model of Extended Hepatectomy.

Herrero A, Prigent J, Lombard C, Rosseels V, Daujat-Chavanieu M, Breckpot K, Najimi M, Deblandre G, Sokal EM.
Cell transplantation.Cell Transplant.2017 Feb 16;26(2):351-364. doi: 10.3727/096368916X692960. Epub 2016 Sep 21.
There is growing evidence that cell therapy constitutes a promising strategy for liver regenerative medicine. In the setting of hepatic cancer treatments, cell therapy could prove a useful therapeutic approach for managing the acute liver failure that occurs following extended hepatectomy. In this s
PMID 27657746