関: B- cell deficiency

WordNet

any of a large variety of proteins normally present in the body or produced in response to an antigen which it neutralizes, thus producing an immune response

PrepTutorEJDIC

〈U〉〈C〉(…の)(量・額などの)不足,欠乏《+『of』(『in』)+『名』》 / 〈C〉不足分,不足量,不足額 / 〈C〉(精神・肉体などの)欠陥
抗体,免疫体,抗毒素

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2016/05/08 12:32:08」(JST)

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Humoral immune deficiencies are conditions which cause impairment of humoral immunity, which can lead to immunodeficiency. It can be mediated by insufficient number or function of B cells, the plasma cells they differentiate into (with these two potentially being caused by B cell lymphocytopenia), or the antibody secreted by the plasma cells.

They are associated with increased vulnerability to infection, but can be difficult to detect (or asymptomatic) in the absence of infection.

They can be associated with increased risk of gastric cancer.^[1]

Causes

One way of classifying humoral immune deficiencies are into primary (or hereditary) versus secondary (or acquired) ones.

Primary

The International Union of Immunological Societies classifies primary immune deficiencies of the humoral system (here termed "Predominantly antibody deficiencies") as follows:^[2]

Absent B cells with a resultant severe reduction of all types of antibody: X-linked agammaglobulinemia (btk deficiency, or Bruton's agammaglobulinemia), μ-Heavy chain deficiency, l 5 deficiency, Igα deficiency, BLNK deficiency, thymoma with immunodeficiency
B cells low but present or normal, but with reduction in 2 or more isotypes (usually IgG & IgA, sometimes IgM): common variable immunodeficiency (CVID), ICOS deficiency, CD19 deficiency, TACI (TNFRSF13B) deficiency, BAFF receptor deficiency.
Normal numbers of B cells with decreased IgG and IgA and increased IgM: Hyper-IgM syndromes
Normal numbers of B cells with isotype or light chain deficiencies: heavy chain deletions, kappa chain deficiency, isolated IgG subclass deficiency, IgA with IgG subsclass deficiency, selective immunoglobulin A deficiency
Specific antibody deficiency to specific antigens with normal B cell and normal Ig concentrations
Transient hypogammaglobulinemia of infancy (THI)

Secondary

Secondary (or acquired) forms of humoral immune deficiency are mainly:^[3]

Multiple myeloma
Chronic lymphoid leukemia
AIDS

Presentation and complications

The signs and symptoms of humoral immune deficiency depend 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.^[4]

References

^ den Hartog G, Jansen JB, van der Meer JW, Lamers CB (1992). "Gastric abnormalities in humoral immune deficiency syndromes". Scand. J. Gastroenterol. Suppl. 194: 38–40. PMID 1298045.
^ Notarangelo L, Casanova JL, Conley ME, et al. (2006). "Primary immunodeficiency diseases: an update from the International Union of Immunological Societies Primary Immunodeficiency Diseases Classification Committee Meeting in Budapest, 2005". J. Allergy Clin. Immunol. 117 (4): 883–96. doi:10.1016/j.jaci.2005.12.1347. PMID 16680902.
^ 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.
^ Immunodeficiency by Dr. Saul Greenberg. University of Toronto. Last updated, on February 5, 2009

UpToDate Contents

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1. 特異的抗体欠損 specific antibody deficiency
2. 原発性の体液性免疫不全：概要 primary humoral immunodeficiencies an overview
3. IgG サブクラス欠乏症 igg subclass deficiency
4. 予防接種に対する免疫応答の評価 assessing the immunologic response to vaccination
5. 新生児期の免疫不全の認識 recognition of immunodeficiency in the newborn period

English Journal

Phagocyte NADPH oxidase and specific immunity.

Cachat J1, Deffert C2, Hugues S1, Krause KH1.
Clinical science (London, England : 1979).Clin Sci (Lond).2015 May 1;128(10):635-48. doi: 10.1042/CS20140635.
The phagocyte NADPH oxidase NOX2 produces reactive oxygen species (ROS) and is a well-known player in host defence. However, there is also increasing evidence for a regulatory role of NOX2 in adaptive immunity. Deficiency in phagocyte NADPH oxidase causes chronic granulomatous disease (CGD) in huma
PMID 25760962

DNA-PKcs deficiency inhibits glioblastoma cell-derived angiogenesis after ionizing radiation.

Liu Y1, Zhang L, Liu Y, Sun C, Zhang H, Miao G, Di CX, Zhou X, Zhou R, Wang Z.
Journal of cellular physiology.J Cell Physiol.2015 May;230(5):1094-103. doi: 10.1002/jcp.24841.
DNA-dependent protein kinase catalytic subunit (DNA-PKcs) plays a critical role in non-homologous end-joining repair of DNA double-strand breaks (DSB) induced by ionizing radiation (IR). Little is known, however, regarding the relationship between DNA-PKcs and IR-induced angiogenesis; thus, in this
PMID 25294801

ZmpTAC12 binds single-stranded nucleic acids and is essential for accumulation of the plastid-encoded polymerase complex in maize.

Pfalz J1, Holtzegel U, Barkan A, Weisheit W, Mittag M, Pfannschmidt T.
The New phytologist.New Phytol.2015 May;206(3):1024-1037. doi: 10.1111/nph.13248. Epub 2015 Jan 19.
The plastid-encoded plastid RNA polymerase (PEP) represents the major transcription machinery in mature chloroplasts. Proteomic studies identified four plastome- and at least ten nuclear-encoded proteins making up this multimeric enzyme. Depletion of single subunits is known to result in strongly di
PMID 25599833

Japanese Journal

血栓性血小板減少性紫斑病に対する血漿交換療法(<特集>血小板減少症とアフェレシス)

上田恭典
日本アフェレシス学会雑誌 31(1), 14-19, 2012-02-29
… Deficiency of ADAMTS13 (a cleaving protease of von Willebrand factor) was demonstrated to be a main pathogenesis of TTP in the 1990s. … Rituximab, a chimeric monoclonal antibody against CD20, has been reported to be a good candidate for a breakthrough for this dilemma. …
NAID 110009327448

分類不能型免疫不全症 Update

森尾友宏
日本臨床免疫学会会誌 = Japanese journal of clinical immunology 35(1), 14-22, 2012-02-28
… Common variable immunodeficiency is one of the most common primary immunodeficiency that is categorized into primary antibody deficiency. …
NAID 10030465505

Myocardial Infarction and Normal Coronary Arteries: The Experience of the Cardiology Department of Sfax, Tunisia

Abid Leila,Bahloul Amine,Frikha Zied,Mallek Souad,Abid Dorra,Akrout Malek,Hentati Mourad,Kammoun Samir
Internal Medicine 51(15), 1959-1967, 2012
… Coagulation Disorders, such as activated protein C resistance (APC) resistance, protein C deficiency and antiphospholipid antibody syndrome were found in 4 of 12 patients who underwent systematic examination. …
NAID 130002062261