関: gammopathy

WordNet

a disturbance in the synthesis of immunoglobulins; proteins having antibody activity increase greatly in the blood

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/10/22 13:25:14」(JST)

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Dysgammaglobulinemia is a type of immune disorder characterized by a reduction in some types of gamma globulins.^[1]^[2]

It is distinguished from hypogammaglobulinemia, which is a reduction in all types of gamma globulins.^[3]

Hyper IgM syndrome can be considered a form of dysgammaglobulinemia, because it results from a failure of transformation from IgM production to production of other antibodies, and so the condition can be interpreted as a reduction of the other types.

References

^ "Dysgammaglobulinemia" at Dorland's Medical Dictionary
^ Dysgammaglobulinemia at eMedicine Dictionary
^ "hypogammaglobulinemia" at Dorland's Medical Dictionary

External links

Raif S. Geha et al.: "Hyper Immunoglobulin M Immunodeficiency (Dysgammaglobulinemia)", Journal of Clinical Investigation, 1979 August; 64(2): 385–391, doi:10.1172/JCI109473. Accessed 2009-07-17.
Andre Cruchaud et al.: "The site of synthesis of the 19S T-globulins in dysgammaglobulinemia" (1962). Accessed 2009-07-17.

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1. 自己免疫性リンパ増殖症候群(ALPS)：臨床的特徴および診断 autoimmune lymphoproliferative syndrome alps clinical features and diagnosis
2. X連鎖リンパ増殖性疾患 x linked lymphoproliferative disease
3. 軟骨毛髪形成不全症 cartilage hair hypoplasia
4. 幼児期の一過性低ガンマグロブリン血症 transient hypogammaglobulinemia of infancy
5. 先天性風疹症候群：臨床的特徴および診断 congenital rubella syndrome clinical features and diagnosis

English Journal

SAP and XIAP deficiency in hemophagocytic lymphohistiocytosis.

Yang X, Miyawaki T, Kanegane H.SourceDepartment of Pediatrics, Graduate School of Medicine and Pharmaceutical Sciences, University of Toyama, Toyama, Japan.
Pediatrics international : official journal of the Japan Pediatric Society.Pediatr Int.2012 Aug;54(4):447-54. doi: 10.1111/j.1442-200X.2012.03683.x.
Hemophagocytic lymphohistiocytosis (HLH) is a multisystem inflammatory disorder due to cytokine overproduction from excessively activated lymphocytes and macrophages. HLH has been divided into two subgroups: primary HLH and secondary HLH. Primary HLH includes PRF1, UNC13D, STX11, STXBP2, RAB27A, LYS
PMID 22672194

SAP expression in invariant NKT cells is required for cognate help to support B-cell responses.

Detre C, Keszei M, Garrido-Mesa N, Kis-Toth K, Castro W, Agyemang AF, Veerapen N, Besra GS, Carroll MC, Tsokos GC, Wang N, Leadbetter EA, Terhorst C.SourceDivision of Immunology, Department of Medicine, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA 02115, USA. cdetre@bidmc.harvard.edu
Blood.Blood.2012 Jul 5;120(1):122-9. Epub 2012 May 21.
One of the manifestations of X-linked lymphoproliferative disease (XLP) is progressive agammaglobulinemia, caused by the absence of a functional signaling lymphocyte activation molecule (SLAM)-associated protein (SAP) in T, invariant natural killer T (NKT) cells and NK cells. Here we report that α-
PMID 22613797

Japanese Journal

Transfer factorにより一過性にIgGの正常化をみたdysgammaglobulinemiaの1例

吉田信之,宮崎澄雄,柴田瑠美子
日本臨床免疫学会会誌 8(2), 106-109, 1985
… Dysgammaglobulinemia type 1は, IgM増加とIgG, IgA著減を呈する免疫不全症である.著者らは1歳男児で気道感染とリンパ節炎を反復する同症例を経験した.血清IgMは正常上限であり, IgG, IgAは著明に低下していた.分泌IgAも検出できなかった.遅延型皮膚反応やリンパ球芽球化反応など細胞性免疫能は正常であった.スルホ化γグロブリンによる補充療法を開始したところ,血清IgMは徐々に低下した.発症した間質性肺炎に対しtransfer factor …
NAID 130000848515