secondary immunodeficiency

続発性免疫不全症

WordNet

1. not of major importance; "played a secondary role in world events"
2. the defensive football players who line up behind the linemen
3. being of second rank or importance or value; not direct or immediate; "the stone will be hauled to a secondary crusher"; "a secondary source"; "a secondary issue"; "secondary streams"
4. belonging to a lower class or rank
5. depending on or incidental to what is original or primary; "a secondary infection"
6. immunological disorder in which some part of the bodys immune system is inadequate and resistance to infectious diseases is reduced

PrepTutorEJDIC

1. 『第2の』,2番目の;(…の)次の《+『to』+『名』》 / 派生的な,二次的な,あまり重要でない / 中等学校[教育]の / 二次電流の

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2014/04/27 06:29:47」(JST)

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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. 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.
• T cell deficiency, often causes secondary disorders such as acquired immune deficiency syndrome.
• 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	Main pathogens of resultant infections
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, and intracellular fungal infections.
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, particularly: Streptococcus pneumoniae Haemophilus influenzae Neisseria meningitidis 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 (PID)

Main article: Primary immunodeficiency

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

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

Further information: Immunosuppression

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), 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.

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 a caused by 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. (heh)

References

External links

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

UpToDate Contents

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• 1. 基礎疾患の状態、環境曝露、およびその他の原因による二次性免疫不全症 secondary immunodeficiency due to underlying disease states environmental exposures and miscellaneous causes
• 2. Secondary immunodeficiency induced by biologic therapies
• 3. 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
• 4. 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
• 5. 高齢者における免疫機能 immune function in older adults

English Journal

• Managing patients with side effects and adverse events to immunoglobulin therapy.

• Azizi G1,2, Abolhassani H2,3, Asgardoon MH2, Shaghaghi S2,4, Negahdari B5, Mohammadi J6, Rezaei N2, Aghamohammadi A2.
• Expert review of clinical pharmacology.Expert Rev Clin Pharmacol.2016 Jan;9(1):91-102. doi: 10.1586/17512433.2016.1105131. Epub 2015 Oct 23.
• Immunoglobulin therapy has not only served as a lifesaving approach for the prevention and treatment of infections in primary and secondary immunodeficiency diseases, but has also been used as an immunomodulatory agent for autoimmune and inflammatory disorders and to provide passive immunity for som
• PMID 26496172

• The use of unboosted darunavir in the setting of ritonavir intolerance: three case reports.

• Smith K, Gomes D, Nixon D, Fulco PP.
• International journal of clinical pharmacology and therapeutics.Int J Clin Pharmacol Ther.2015 Dec 4. [Epub ahead of print]
• OBJECTIVE: To describe three patients with human immunodeficiency virus (HIV) who were successfully managed with unboosted darunavir (uDRV).CASE SUMMARIES: The three cases included one woman and two men aged 58, 54, and 51 years, respectively. All patients were HIV positive and unable to tolerate ri
• PMID 26636419

• Discontinuing Pneumocystis jirovecii Pneumonia Prophylaxis in HIV-Infected Patients With a CD4 Cell Count <200 cells/mm3.

• Sidhu VK1, Foisy MM2, Hughes CA3.
• The Annals of pharmacotherapy.Ann Pharmacother.2015 Dec;49(12):1343-8. doi: 10.1177/1060028015605113. Epub 2015 Sep 10.
• OBJECTIVE: To review the evidence for discontinuing primary and secondary Pneumocystis jirovecii pneumonia (PJP) prophylaxis in HIV-infected patients with a CD4 count <200 cells/mm(3).DATA SOURCES: We conducted a literature search in MEDLINE, EMBASE, Cochrane Library, Google Scholar, and the Inte
• PMID 26358129

Japanese Journal

• Post-marketing observational study on 5% intravenous immunoglobulin therapy in patients with secondary immunodeficiency and recurrent serious bacterial infections

• Gunther Georg,Dreger Bettina
• Microbiology and immunology 57(7), 527-535, 2013-07
• NAID 40019719188

• 原発性免疫不全症候群関連の免疫性血小板減少症 (第74回日本血液学会学術集会) -- (シンポジウム1 The Research Front Line of Immune Thrombocytopenia)

• 高木 正稔,今井 耕介,森尾 友宏 [他]
• 臨床血液 54(4), 357-364, 2013-04
• NAID 40019681065

• Dialysis Purification of Integrase-DNA Complexes Provides High-Resolution Atomic Force Microscopy Images: Dimeric Recombinant HIV-1 Integrase Binding and Specific Looping on DNA.

• Tsuruyama Tatsuaki,Nakai Tonau,Ohmori Rei,Ozeki Munetaka,Tamaki Keiji,Yoshikawa Kenichi
• PloS one 8(1), 2013-01-14
• … Our resulting atomic force microscopy analyses indicated precise size of binding human immunodeficiency virus type 1 (HIV-1) recombinant integrase in a tetrameric manner, inducing formation of a loop-like or figure-eight-like secondary structure in the target DNA. … Our findings regarding the target DNA secondary structure provide new insights into the intermediate states of retroviral integration. …
• NAID 120005241337

Related Links

• Secondary immunodeficiency - RightDiagnosis.com

List of 10 disease causes of Secondary immunodeficiency, patient stories, diagnostic guides. Diagnostic checklist, medical tests, doctor questions, and related signs or symptoms for Secondary immunodeficiency. ... Secondary ...

• Immunodeficiency (Primary and Secondary). Information. | Patient

Immunodeficiency classified as primary and secondary. Immunodeficiency (Primary and Secondary) are mostly inherited single-gene disorders. More about Immunodeficiency. ... Secondary immunodeficiencies There are many ...

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リンク元	「続発性免疫不全症」
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関連記事	「secondary」「immunodeficiency」

「続発性免疫不全症」

　　[★]

英

secondary immunodeficiency

「secondary immunodeficiency diseases」

　　[★]

関

specific diseases/disorders

関

specific diseases/disorders

「secondary」

　　[★]

• adj.

• (地位・価値・重要性などが)第2の、2番目の、(～の)次の(to)。二次的な、従位の

Postmortem examination of patients with such obstructions without a history of clinical manifestations of myocardial ischemia often shows macroscopic scars secondary to MI in regions supplied by diseased coronary arteries, with or without collateral circulation.

in the past the commonest cause of death has been venous thrombosis followed by infection secondary to severe neutropenia and hemorrhage secondary to severe thrombocytopenia.(HIM.661)(重度の好中球減少に次ぐ感染と血小板減少に次ぐ出血に続発する静脈血栓症が昔の最も一般的な死因であった)

• 副次的な(発生などが)派生的な

　 　 　 　 　 　

「immunodeficiency」

　　[★] 免疫不全症 → 免疫不全症候群