- 関
- B-cell deficiency
WordNet
- secure against; "immune from taxation as long as he resided in Bermuda"; "immune from criminal prosecution"
- a person who is immune to a particular infection
- relating to or conferring immunity (to disease or infection) (同)resistant
- (usually followed by `to'
- relating to the condition of immunity; "the immune system"
- put into a good mood (同)humour
- the trait of appreciating (and being able to express) the humorous; "she didnt appreciate my humor"; "you cant survive in the army without a sense of humor" (同)humour, sense of humor, sense of humour
- (Middle Ages) one of the four fluids in the body whose balance was believed to determine your emotional and physical state; "the humors are blood and phlegm and yellow and black bile" (同)humour
- the quality of being funny; "I fail to see the humor in it" (同)humour
- of or relating to bodily fluids
PrepTutorEJDIC
- 免疫の / 《補語にのみ用いて》(影響・攻撃・義務・税などを)免れている《+『against』(『from, to』)+『名』》
- 〈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
Wikipedia preview
出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2013/04/24 13:44:28」(JST)
[Wiki en表示]
| Humoral immune deficiency |
| Classification and external resources |
B cells and antibody |
| ICD-10 |
D80 |
| ICD-9 |
279.0 |
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]
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Contents
- 1 Causes
- 1.1 Primary
- 1.2 Secondary
- 2 Presentation and complications
- 3 References
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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
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Immune disorders: Lymphoid and complement immunodeficiency (D80–D85, 279.0–4)
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| Primary |
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Antibody/humoral (B)
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Hypogammaglobulinemia
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- X-linked agammaglobulinemia
- Transient hypogammaglobulinemia of infancy
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Dysgammaglobulinemia
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- IgA deficiency
- IgG deficiency
- IgM deficiency
- Hyper IgM syndrome (2
- 3
- 4
- 5)
- Wiskott-Aldrich syndrome
- Hyper-IgE syndrome
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Other
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- Common variable immunodeficiency
- ICF syndrome
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T cell deficiency (T)
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- thymic hypoplasia: hypoparathyroid (Di George's syndrome)
- euparathyroid (Nezelof syndrome
- Ataxia telangiectasia)
peripheral: Purine nucleoside phosphorylase deficiency
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Severe combined (B+T)
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- x-linked: X-SCID
autosomal: Adenosine deaminase deficiency
- Omenn syndrome
- ZAP70 deficiency
- Bare lymphocyte syndrome
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| Acquired |
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Leukopenia:
Lymphocytopenia |
- Idiopathic CD4+ lymphocytopenia
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| Complement deficiency |
- C1-inhibitor (Angioedema/Hereditary angioedema)
- Complement 2 deficiency/Complement 4 deficiency
- MBL deficiency
- Properdin deficiency
- Complement 3 deficiency
- Terminal complement pathway deficiency
- Paroxysmal nocturnal hemoglobinuria
- Complement receptor deficiency
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cell/phys/auag/auab/comp, igrc
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UpToDate Contents
全文を閲覧するには購読必要です。 To read the full text you will need to subscribe.
English Journal
- CCR6-Dependent Positioning of Memory B Cells Is Essential for Their Ability To Mount a Recall Response to Antigen.
- Elgueta R1, Marks E2, Nowak E3, Menezes S4, Benson M3, Raman VS3, Ortiz C4, O'Connell S4, Hess H5, Lord GM2, Noelle R6.
- Journal of immunology (Baltimore, Md. : 1950).J Immunol.2015 Jan 15;194(2):505-13. doi: 10.4049/jimmunol.1401553. Epub 2014 Dec 12.
- Chemokine-dependent localization of specific B cell subsets within the immune microarchitecture is essential to ensure successful cognate interactions. Although cognate interactions between T cells and memory B cells (Bmem) are essential for the secondary humoral immune responses, the chemokine resp
- PMID 25505290
- Cdc42 is a key regulator of B cell differentiation and is required for antiviral humoral immunity.
- Burbage M1, Keppler SJ1, Gasparrini F1, Martínez-Martín N1, Gaya M1, Feest C1, Domart MC1, Brakebusch C2, Collinson L1, Bruckbauer A1, Batista FD3.
- The Journal of experimental medicine.J Exp Med.2015 Jan 12;212(1):53-72. doi: 10.1084/jem.20141143. Epub 2014 Dec 29.
- The small Rho GTPase Cdc42, known to interact with Wiskott-Aldrich syndrome (WAS) protein, is an important regulator of actin remodeling. Here, we show that genetic ablation of Cdc42 exclusively in the B cell lineage is sufficient to render mice unable to mount antibody responses. Indeed Cdc42-defic
- PMID 25547673
- Fertility, Pregnancies and Outcomes Reported by Females with Common Variable Immune Deficiency and Hypogammaglobulinemia: Results from an Internet-Based Survey.
- Gundlapalli AV1, Scalchunes C, Boyle M, Hill HR.
- Journal of clinical immunology.J Clin Immunol.2015 Jan 9. [Epub ahead of print]
- BACKGROUND: Issues of fertility and pregnancy place an extra burden on females with primary immunodeficiencies. Patients lack reliable information and providers lack guidelines to counsel patients on these anxiety-provoking matters.OBJECTIVE: To collate concerns and experiences related to fertility
- PMID 25572592
Japanese Journal
- Mucosal Immune Responses in W/Wv and Sl/Sld Mutant Mice
- Yoshino Naoto,Kanno Hiroyuki,Takahashi Kiyomi,Endo Masahiro,Sato Shigehiro
- Experimental Animals 61(4), 407-416, 2012
- … In order to identify potential unanticipated side reactions and immune responses, the evaluation of candidate vaccines should include immunization of the murine model of the disease in question and mutant animals, as well as normal laboratory animals. … We employed WBB6F1-W/Wv and WBB6F1-Sl/Sld mutant mice, which are genetically mast cell deficient and lack intestinal pacemaker activity due to a severe deficiency in interstitial cells of Cajal. …
- NAID 130001893566
- 新しいガイドラインにおける免疫不全に伴う肺炎の考え方 (特集 日本呼吸器学会院内肺炎の新しいガイドライン)
- 高橋 秀実
- 生物物理 37(3), 111-115, 1997-06-25
- … As the major targets of immunity have been changing from extracellular antigens, such as hafmful bacteria and their toxins, to intracellular events caused by viral genes or oncogenes, the main stream of immunology appears to have been shifted from humoral arm to cellular arm. … In this review, by modeling human immune deficiency virus (HIV) envelope protein, possible molecular control of cellular immune responses to viral components will be discussed. …
- NAID 110001157454
Related Links
- WikiDoc Resources for Humoral immune deficiency Articles Most recent articles on Humoral immune deficiency Most cited articles on Humoral immune deficiency Review articles on Humoral immune deficiency Articles on Humoral ...
- ^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 ...
★リンクテーブル★
[★]
- 不足、欠乏、欠失、欠如、欠損、不十分。栄養不足、栄養素欠乏、欠乏症。(遺伝子)(染色体内の)遺伝子欠失
- 欠けているもの、不足している物。不足分。不完全なもの、欠点のあるもの
- 関
- absence, agenesis, dearth, defect, defective, deficient, deficit, delete, deletion, deletional, depletion, deprivation, deprive, lack, miss, missing, morphological defect, paucity, scarce, scarcity, starve
[★]
- 関
- immunisation、immunise、immunity、immunization、immunize、immuno、immunologic
[★]
- おかしみ、ユーモア
- 気質
- 気分、気持ち
- (古)液、(中世医学)体液
[★]
免疫不全症候群
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- ies