- 同
- CD43抗原, leukosialin,
- ウィスコット・オールドリッチ症候群タンパク質、ウィスコット・アルドリッチ症候群タンパク質
- Wiskott-Aldrich syndrome protein, WASP, Wiskott-Aldrich症候群タンパク質
発現細胞
- leukocytes, except resting B cells
- 胸腺細胞、T細胞、活性化B細胞、形質細胞、NK細胞、好中球、単球、マクロファージ、血小板、stem cell(WCH.49)
分子量
- 115-135kDa(好中球)
- 95-115(T細胞)
機能
- has extended structure, approx. 45nm long and may be anti-adhesive
別名
ファミリー
- 同
- Leukosialin, Sialophorin, Wiskott-Aldrich syndrome protein
- 同
- leukosialin&B-cell development
PrepTutorEJDIC
- certificate of deposit / (また『C.D.』)Civil Defense民間防衛
Wikipedia preview
出典(authority):フリー百科事典『ウィキペディア(Wikipedia)』「2014/11/10 14:24:27」(JST)
[Wiki en表示]
Sialophorin |
Identifiers |
Symbols |
SPN ; CD43; GALGP; GPL115; LSN |
External IDs |
OMIM: 182160 MGI: 98384 HomoloGene: 36108 GeneCards: SPN Gene |
Gene ontology |
Molecular function |
• transmembrane signaling receptor activity
|
Cellular component |
• uropod
• basement membrane
• extracellular space
• plasma membrane
• integral component of plasma membrane
• external side of plasma membrane
• cell surface
• membrane
• extracellular vesicular exosome
|
Biological process |
• response to protozoan
• negative regulation of type IV hypersensitivity
• chemotaxis
• immune response
• cellular defense response
• negative regulation of cell adhesion
• establishment or maintenance of cell polarity
• signal transduction
• cell surface receptor signaling pathway
• blood coagulation
• T cell costimulation
• positive regulation of T cell proliferation
• negative regulation of T cell proliferation
• positive regulation of tumor necrosis factor biosynthetic process
• defense response to bacterium
• negative thymic T cell selection
• regulation of defense response to virus
• leukocyte migration
• apoptotic signaling pathway
|
Sources: Amigo / QuickGO |
|
Orthologs |
Species |
Human |
Mouse |
|
Entrez |
6693 |
20737 |
|
Ensembl |
ENSG00000197471 |
ENSMUSG00000051457 |
|
UniProt |
P16150 |
P15702 |
|
RefSeq (mRNA) |
NM_001030288 |
NM_001037810 |
|
RefSeq (protein) |
NP_001025459 |
NP_001032899 |
|
Location (UCSC) |
Chr 16:
29.67 – 29.68 Mb |
Chr 7:
127.13 – 127.14 Mb |
|
PubMed search |
[1] |
[2] |
|
|
Leukosialin also known as sialophorin or CD43 (cluster of differentiation 43) is a transmembrane cell surface protein that in humans is encoded by the SPN (sialophorin) gene.[1][2][3]
Contents
- 1 Function
- 2 Clinical significance
- 3 Interactions
- 4 References
- 5 Further reading
- 6 External links
Function
Sialophorin (leukosialin) is a major sialoglycoprotein on the surface of human T lymphocytes, monocytes, granulocytes, and some B lymphocytes, which appears to be important for immune function and may be part of a physiologic ligand-receptor complex involved in T-cell activation.[1]
Clinical significance
Defects in the CD43 molecule are associated with the development of Wiskott-Aldrich syndrome.[4] It also appears in about 25% of intestinal MALTomas.[citation needed] Using immunohistochemistry, CD43 can be demonstrated in the paracortical T-cells of healthy lymph nodes and tonsils; it is also positive in a range of lymphoid and myeloid tumours. Although it is present in over 90% of T-cell lymphomas, it is generally less effective at demonstrating this condition than is CD3 antigen. However, it may be useful as part of a panel to demonstrate B-cell lymphoblastic lymphoma, since the malignant cells in this condition are often CD43 positive, and may be difficult to stain with other antibodies. Because it stains granulocytes and their precursors, it is also an effective marker for myeloid tumours.[5]
Interactions
CD43 has been shown to interact with EZR[6] and Moesin.[6][7]
References
- ^ a b "Entrez Gene: SPN sialophorin (leukosialin, CD43)".
- ^ Pallant A, Eskenazi A, Mattei MG, Fournier RE, Carlsson SR, Fukuda M, Frelinger JG (February 1989). "Characterization of cDNAs encoding human leukosialin and localization of the leukosialin gene to chromosome 16". Proc. Natl. Acad. Sci. U.S.A. 86 (4): 1328–32. Bibcode:1989PNAS...86.1328P. doi:10.1073/pnas.86.4.1328. PMC 286683. PMID 2521952.
- ^ Shelley CS, Remold-O'Donnell E, Davis AE, Bruns GA, Rosen FS, Carroll MC, Whitehead AS (April 1989). "Molecular characterization of sialophorin (CD43), the lymphocyte surface sialoglycoprotein defective in Wiskott-Aldrich syndrome". Proc. Natl. Acad. Sci. U.S.A. 86 (8): 2819–23. Bibcode:1989PNAS...86.2819S. doi:10.1073/pnas.86.8.2819. PMC 287010. PMID 2784859.
- ^ Remold-O'Donnell E, Rosen FS (1990). "Sialophorin (CD43) and the Wiskott-Aldrich syndrome". Immunodefic Rev 2 (2): 151–74. PMID 2223062.
- ^ Leong, Anthony S-Y; Cooper, Kumarason; Leong, F Joel W-M (2003). Manual of Diagnostic Cytology (2 ed.). Greenwich Medical Media, Ltd. p. 113. ISBN 1-84110-100-1.
- ^ a b Serrador JM, Nieto M, Alonso-Lebrero JL, del Pozo MA, Calvo J, Furthmayr H, Schwartz-Albiez R, Lozano F, González-Amaro R, Sánchez-Mateos P, Sánchez-Madrid F (June 1998). "CD43 interacts with moesin and ezrin and regulates its redistribution to the uropods of T lymphocytes at the cell-cell contacts". Blood 91 (12): 4632–44. PMID 9616160.
- ^ Yonemura S, Hirao M, Doi Y, Takahashi N, Kondo T, Tsukita S, Tsukita S (February 1998). "Ezrin/radixin/moesin (ERM) proteins bind to a positively charged amino acid cluster in the juxta-membrane cytoplasmic domain of CD44, CD43, and ICAM-2". J. Cell Biol. 140 (4): 885–95. doi:10.1083/jcb.140.4.885. PMC 2141743. PMID 9472040.
Further reading
- Fukuda M, Carlsson SR (1987). "Leukosialin, a major sialoglycoprotein on human leukocytes as differentiation antigens.". Med. Biol. 64 (6): 335–43. PMID 2950285.
- Rogaev EI, Keryanov SA (1993). "Unusual variability of the complex dinucleotide repeat block at the SPN locus.". Hum. Mol. Genet. 1 (8): 657. doi:10.1093/hmg/1.8.657. PMID 1301183.
- Rosenstein Y, Park JK, Hahn WC, et al. (1992). "CD43, a molecule defective in Wiskott-Aldrich syndrome, binds ICAM-1.". Nature 354 (6350): 233–5. Bibcode:1991Natur.354..233R. doi:10.1038/354233a0. PMID 1683685.
- Schmid K, Hediger MA, Brossmer R, et al. (1992). "Amino acid sequence of human plasma galactoglycoprotein: identity with the extracellular region of CD43 (sialophorin).". Proc. Natl. Acad. Sci. U.S.A. 89 (2): 663–7. Bibcode:1992PNAS...89..663S. doi:10.1073/pnas.89.2.663. PMC 48299. PMID 1731338.
- Kudo S, Fukuda M (1991). "A short, novel promoter sequence confers the expression of human leukosialin, a major sialoglycoprotein on leukocytes.". J. Biol. Chem. 266 (13): 8483–9. PMID 1827122.
- Park JK, Rosenstein YJ, Remold-O'Donnell E, et al. (1991). "Enhancement of T-cell activation by the CD43 molecule whose expression is defective in Wiskott-Aldrich syndrome.". Nature 350 (6320): 706–9. Bibcode:1991Natur.350..706P. doi:10.1038/350706a0. PMID 2023632.
- Shelley CS, Remold-O'Donnell E, Rosen FS, Whitehead AS (1990). "Structure of the human sialophorin (CD43) gene. Identification of features atypical of genes encoding integral membrane proteins.". Biochem. J. 270 (3): 569–76. PMC 1131770. PMID 2241892.
- Srinivas RV, Su T, Trimble LA, et al. (1996). "Enhanced susceptibility to human immunodeficiency virus infection in CD4+ T lymphocytes genetically deficient in CD43.". AIDS Res. Hum. Retroviruses 11 (9): 1015–21. doi:10.1089/aid.1995.11.1015. PMID 8554898.
- Hirao M, Sato N, Kondo T, et al. (1996). "Regulation mechanism of ERM (ezrin/radixin/moesin) protein/plasma membrane association: possible involvement of phosphatidylinositol turnover and Rho-dependent signaling pathway.". J. Cell Biol. 135 (1): 37–51. doi:10.1083/jcb.135.1.37. PMC 2121020. PMID 8858161.
- Yonemura S, Hirao M, Doi Y, et al. (1998). "Ezrin/radixin/moesin (ERM) proteins bind to a positively charged amino acid cluster in the juxta-membrane cytoplasmic domain of CD44, CD43, and ICAM-2.". J. Cell Biol. 140 (4): 885–95. doi:10.1083/jcb.140.4.885. PMC 2141743. PMID 9472040.
- Pedraza-Alva G, Mérida LB, Burakoff SJ, Rosenstein Y (1998). "T cell activation through the CD43 molecule leads to Vav tyrosine phosphorylation and mitogen-activated protein kinase pathway activation.". J. Biol. Chem. 273 (23): 14218–24. doi:10.1074/jbc.273.23.14218. PMID 9603925.
- Serrador JM, Nieto M, Alonso-Lebrero JL, et al. (1998). "CD43 interacts with moesin and ezrin and regulates its redistribution to the uropods of T lymphocytes at the cell-cell contacts.". Blood 91 (12): 4632–44. PMID 9616160.
- Pace KE, Lee C, Stewart PL, Baum LG (1999). "Restricted receptor segregation into membrane microdomains occurs on human T cells during apoptosis induced by galectin-1.". J. Immunol. 163 (7): 3801–11. PMID 10490978.
- Seveau S, Keller H, Maxfield FR, et al. (2000). "Neutrophil polarity and locomotion are associated with surface redistribution of leukosialin (CD43), an antiadhesive membrane molecule.". Blood 95 (8): 2462–70. PMID 10753822.
- Fratazzi C, Manjunath N, Arbeit RD, et al. (2000). "A macrophage invasion mechanism for mycobacteria implicating the extracellular domain of CD43.". J. Exp. Med. 192 (2): 183–92. doi:10.1084/jem.192.2.183. PMC 2193255. PMID 10899905.
- Santana MA, Pedraza-Alva G, Olivares-Zavaleta N, et al. (2000). "CD43-mediated signals induce DNA binding activity of AP-1, NF-AT, and NFkappa B transcription factors in human T lymphocytes.". J. Biol. Chem. 275 (40): 31460–8. doi:10.1074/jbc.M005231200. PMID 10908570.
- van den Berg TK, Nath D, Ziltener HJ, et al. (2001). "Cutting edge: CD43 functions as a T cell counterreceptor for the macrophage adhesion receptor sialoadhesin (Siglec-1).". J. Immunol. 166 (6): 3637–40. doi:10.4049/jimmunol.166.6.3637. PMID 11238599.
External links
- CD43 antigen at the US National Library of Medicine Medical Subject Headings (MeSH)
Protein, glycoconjugate: glycoproteins and glycopeptides
|
|
Mucoproteins |
Mucin
|
- CD43
- CD164
- MUC1
- MUC2
- MUC3A
- MUC3B
- MUC4
- MUC5AC
- MUC5B
- MUC6
- MUC7
- MUC8
- MUC12
- MUC13
- MUC15
- MUC16
- MUC17
- MUC19
- MUC20
|
|
Other
|
- Haptoglobin
- Intrinsic factor
- Orosomucoid
- Peptidoglycan
- Phytohaemagglutinin
- Ovomucin
|
|
|
Proteoglycans |
CS/DS
|
- Decorin
- Biglycan
- Versican
|
|
HS/CS
|
|
|
CS
|
- Chondroitin sulfate proteoglycans: Aggrecan
- Neurocan
- Brevican
- CD44
- CSPG4
- CSPG5
- Platelet factor 4
- Structural maintenance of chromosomes 3
|
|
KS
|
- Fibromodulin
- Lumican
- Keratocan
|
|
HS
|
|
|
|
Other |
- Activin and inhibin
- ADAM
- Alpha 1-antichymotrypsin
- Apolipoprotein H
- CD70
- Asialoglycoprotein
- Avidin
- B-cell activating factor
- 4-1BB ligand
- Cholesterylester transfer protein
- Clusterin
- Colony-stimulating factor
- Hemopexin
- Lactoferrin
- Membrane glycoproteins
- Myelin protein zero
- Osteonectin
- Protein C
- Protein S
- Serum amyloid P component
- Sialoglycoprotein
- CD43
- Glycophorin
- Glycophorin C
- Thrombopoietin
- Thyroglobulin
- Thyroxine-binding proteins
- Transcortin
- Tumor necrosis factor alpha
- Uteroglobin
- Vitronectin
|
|
|
mt, k, c/g/r/p/y/i, f/h/s/l/o/e, a/u, n, m
|
k, cgrp/y/i, f/h/s/l/o/e, au, n, m, epon
|
m (A16/C10), i (k, c/g/r/p/y/i, f/h/s/o/e, a/u, n, m)
|
|
- Biochemical families
- carbohydrates
- alcohols
- glycoproteins
- glycosides
- lipids
- eicosanoids
- fatty acids / intermediates
- glycerides
- phospholipids
- sphingolipids
- steroids
- nucleic acids
- constituents / intermediates
- proteins
- amino acids / intermediates
- tetrapyrroles / intermediates
|
|
Protein, glycoconjugate: glycoproteins and glycopeptides
|
|
Mucoproteins |
Mucin
|
- CD43
- CD164
- MUC1
- MUC2
- MUC3A
- MUC3B
- MUC4
- MUC5AC
- MUC5B
- MUC6
- MUC7
- MUC8
- MUC12
- MUC13
- MUC15
- MUC16
- MUC17
- MUC19
- MUC20
|
|
Other
|
- Haptoglobin
- Intrinsic factor
- Orosomucoid
- Peptidoglycan
- Phytohaemagglutinin
- Ovomucin
|
|
|
Proteoglycans |
CS/DS
|
- Decorin
- Biglycan
- Versican
|
|
HS/CS
|
|
|
CS
|
- Chondroitin sulfate proteoglycans: Aggrecan
- Neurocan
- Brevican
- CD44
- CSPG4
- CSPG5
- Platelet factor 4
- Structural maintenance of chromosomes 3
|
|
KS
|
- Fibromodulin
- Lumican
- Keratocan
|
|
HS
|
|
|
|
Other |
- Activin and inhibin
- ADAM
- Alpha 1-antichymotrypsin
- Apolipoprotein H
- CD70
- Asialoglycoprotein
- Avidin
- B-cell activating factor
- 4-1BB ligand
- Cholesterylester transfer protein
- Clusterin
- Colony-stimulating factor
- Hemopexin
- Lactoferrin
- Membrane glycoproteins
- Myelin protein zero
- Osteonectin
- Protein C
- Protein S
- Serum amyloid P component
- Sialoglycoprotein
- CD43
- Glycophorin
- Glycophorin C
- Thrombopoietin
- Thyroglobulin
- Thyroxine-binding proteins
- Transcortin
- Tumor necrosis factor alpha
- Uteroglobin
- Vitronectin
|
|
|
mt, k, c/g/r/p/y/i, f/h/s/l/o/e, a/u, n, m
|
k, cgrp/y/i, f/h/s/l/o/e, au, n, m, epon
|
m (A16/C10), i (k, c/g/r/p/y/i, f/h/s/o/e, a/u, n, m)
|
|
- Biochemical families
- carbohydrates
- alcohols
- glycoproteins
- glycosides
- lipids
- eicosanoids
- fatty acids / intermediates
- glycerides
- phospholipids
- sphingolipids
- steroids
- nucleic acids
- constituents / intermediates
- proteins
- amino acids / intermediates
- tetrapyrroles / intermediates
|
|
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
- Orbital and ocular adnexal Mucosa-Associated Lymphoid Tissue (MALT) lymphomas: a single-center 10-year experience.
- Smiljanic M, Milosevic R, Antic D, Andjelic B, Djurasinovic V, Todorovic M, Bila J, Bogdanovic A, Mihaljevic B.SourceClinic of Hematology, Clinical Centre of Serbia, Belgrade, Serbia, mikesmiljanic79@gmail.com.
- Medical oncology (Northwood, London, England).Med Oncol.2013 Dec;30(4):722. doi: 10.1007/s12032-013-0722-5. Epub 2013 Sep 13.
- Orbital and ocular andexal Mucosa-Associated Lymphoid Tissue Lymphoma (MALT) or ocular adnexal MALT lymphoma (OAML) is the most common of all eye non-Hodgkin lymphomas. Autoimmune inflammatory disorders and chronic infections are important etiological factors and CD5 and CD43 (sialophorin) tumor mar
- PMID 24026660
- Transplantation of mesenchymal stem cells into the skeletal muscle induces cytokine generation.
- Gala K, Burdzińska A, Idziak M, Wilczek E, Pączek L.SourceDepartment of Immunology, Transplantology and Internal Diseases, Transplantation Institute, Medical University of Warsaw, Poland. Electronic address: kamila.gala@wum.edu.pl.
- Cytokine.Cytokine.2013 Oct;64(1):243-50. doi: 10.1016/j.cyto.2013.06.314. Epub 2013 Jul 13.
- Mesenchymal stem cells due to the high proliferative potential, capacity of multilineage differentiation became a hope of regenerative medicine. However, the organism's response to the transplantation of MSCs is not fully elucidated. The aim of the present study was to evaluate the acute local tissu
- PMID 23859809
- Inhibition of Mucin-Type O-Glycosylation through Metabolic Processing and Incorporation of N-Thioglycolyl-d-galactosamine Peracetate (Ac5GalNTGc).
- Agarwal K, Kaul R, Garg M, Shajahan A, Jha SK, Sampathkumar SG.SourceLaboratory of Chemical Glycobiology (CGB), National Institute of Immunology (NII) , Aruna Asaf Ali Marg, New Delhi 110067, India.
- Journal of the American Chemical Society.J Am Chem Soc.2013 Sep 25;135(38):14189-97. doi: 10.1021/ja405189k. Epub 2013 Sep 16.
- Mucin-type O-glycans form one of the most abundant and complex post-translational modifications (PTM) on cell surface proteins that govern adhesion, migration, and trafficking of hematopoietic cells. Development of targeted approaches to probe functions of O-glycans is at an early stage. Among sever
- PMID 23987472
- Pneumococcal polysaccharide vaccination induces polysaccharide-specific B cells in adult peripheral blood expressing CD19(+)CD20(+)CD3(-)CD70(-)CD27(+)IgM(+)CD43(+)CD5(+/-).
- Leggat DJ, Khaskhely NM, Iyer AS, Mosakowski J, Thompson RS, Weinandy JD, Westerink MA.SourceDepartment of Medicine, University of Toledo, Toledo, OH 43614, United States.
- Vaccine.Vaccine.2013 Sep 23;31(41):4632-40. doi: 10.1016/j.vaccine.2013.07.030. Epub 2013 Aug 1.
- Pneumococcal polysaccharide vaccines have been used to elicit a protective anti-pneumococcal polysaccharide antibody response against Streptococcus pneumoniae in healthy individuals. Identifying human B cells which respond to T-cell independent type-2 antigens, such as pneumococcal polysaccharides,
- PMID 23911852
Japanese Journal
- Macrophage Recognition of Thiol-Group Oxidized Cells : Recognition of Carbohydrate Chains by Macrophage Surface Nucleolin as Apoptotic Cells
- MIKI Yuichi,HIRANO Kazuya,BEPPU Masatoshi
- Bioscience, Biotechnology, and Biochemistry 76(11), 2068-2074, 2012-11
- NAID 40019498903
- Clearance of CD43-Capped Cells by Macrophages: Capping Alone Leads to Phagocytosis
- Oguri Emiri,Miki Yuichi,Hirano Kazuya,Yamanaka Masahiro,Beppu Masatoshi
- Biological and Pharmaceutical Bulletin 35(4), 551-558, 2012
- … In some cases this is achieved via CD43-capped membrane glycoproteins, the sialylpolylactosaminyl chains of which serve as ligands for phagocytosis by macrophages. … Here, we examined one clearance mechanism in detail and determined that capping of CD43 alone is sufficient to initiate phagocytosis. … We induced macrophage-mediated phagocytosis by using cytochalasin B to artificially cap CD43 on healthy (non-apoptotic) Jurkat cells. …
- NAID 130001872204
- Russell 小体の出現を伴った皮膚原発 marginal zone B-cell lymphoma の1例
- 青木 見佳子,川名 誠司,新井 栄一
- Skin cancer : official organ of the Japanese Society for Skin Cancer = 皮膚悪性腫瘍研究会機関誌 25(2), 168-172, 2010-09-15
- … 浸潤細胞はCD20,CD79a,bcl-2,CD43陽性のリンパ球で二次濾胞も形成していた。 …
- NAID 10027731645
Related Pictures
★リンクテーブル★
[★]
- 英
- chronic lymphocytic leukemia, chronic lymphoid leukemia, CLL
- 関
- 白血病
- first aid step1 2006 p.300,302,303,304,309,310,427,430
概念
- the accumulation of nonproliferating mature-appearing lymphocytes in the blood, marrow, lymph nodes, and spleen.
- monoclonal B lymphocytes.
- CD5+ and CD23+CD5, CD23
- derived from memory B cell.
細胞表面マーカー WCH.2438
臨床像
- HIM.693
|
年齢
|
子供における頻度
|
男性(%)
|
ステージI,II vs III,IV(%)
|
B症状(%)
|
骨髄浸潤(%)
|
消化管浸潤(%)
|
5年生存率(%)
|
B細胞CLL/小リンパ球性リンパ腫
|
65
|
まれ
|
53
|
9 vs 91
|
33
|
72
|
3
|
51
|
疫学
- YN.G-49
- 全白血病の2-3%
- 60歳以上に多い。
- 男女比=2:1
病態
- YN.G-49
- 正常な免疫グロブリンの産生が抑制されるばかりでなく、異常な自己抗体を産生 → 続発性免疫不全症、自己免疫性溶血性貧血
USMLE
参考
- 1. [charged] Epidemiology and clinical manifestations of chronic lymphocytic leukemia - uptodate [1]
- 2. [charged] Pathologic features, diagnosis, and differential diagnosis of chronic lymphocytic leukemia - uptodate [2]
- 3. [charged] Pathophysiology and cytogenetics of chronic lymphocytic leukemia - uptodate [3]
[show details]
[★]
- 英
- malignant lymphoma
- ラ
- lymphoma malignum
- 関
悪性リンパ腫とマーカー
- 悪性リンパ腫.xls
|
sIg
|
CD5
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CD10
|
CD19
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CD20
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CD23
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CD43
|
bcl
|
cyclin
|
TdT
|
その他
|
転座
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小リンパ性リンパ腫 small lymphocytic lymphoma 慢性リンパ性白血病 chronic lymphocytic leukemia
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+
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+
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+
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+
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+
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-
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-
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-
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濾胞性リンパ腫 FL
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+
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-
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+
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+
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bcl2 +
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-
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MALTリンパ腫
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-
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-
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+
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マントル細胞リンパ腫 MCL
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+
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+
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-
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+
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+
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びまん性大細胞性B細胞リンパ腫 DLBL
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+
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-
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+/-
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+
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+
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bcl6 +
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前駆Bリンパ芽球性リンパ腫 急性Bリンパ球性白血病 LBL/ALL
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-
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+
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+
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バーキットリンパ腫 BL
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+
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-
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+
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+
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+
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Myc, Ki-67
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t(8,14)Myc;IgH ~80% t(2,8)κ;Myc ~15%
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ホジキンリンパ腫
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CD15, CD30, CD45 -
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t(8,22)Myc;λ ~10%
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成人T細胞白血病 ATL
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CD2, CD3, CD4, CD25, HLA-DR, CD8 -
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病期
参考
- http://www.rinpashu.com/inspection/
国試
[★]
- 英
- Wiskott-Aldrich syndrome, WAS
- 同
- ウィスコット・オールドリッチ症候群, ウィスコット・アルドリック症候群, ウィスコット・アルドリッヒ症候群, Wiskott-Aldrich症候群
- アルドリッチ症候群 オールドリッチ症候群、オールドリッチ症候群、オルドリック症候群、Aldrich症候群、Aldrich syndrome
- 関
- 血小板減少、免疫不全症候群、原発性免疫不全症候群
概念
- 血小板減少と湿疹を伴う免疫不全症
- B細胞-活性化↓
- 男児のみ
- 免疫不全症、血小板減少、湿疹
遺伝
病因
- Xp11.22:WASP遺伝子 (WCH.49によれば、Xp 11-23)の異常による。
病態
- 細胞骨格となるアクチンの再構築に関与している。この機能が損なわれることで免疫シナプスの形成に打撃を与える。
症状
- 1) recurrent infefction 免疫不全 細胞性免疫、IgE高値
- 2) thrombocytepenia 出血性素因 血小板減少による、血便。進行性。
- 3) dermatitis アトピー性皮膚炎 難治性湿疹
- 4) 自己免疫疾患:40-70%の患者で報告がある (参考1)
- 5) 悪性腫瘍:小児でも起こりうるが、思春期と若年男性に好発 (参考1)
免疫不全
- Triad of symptoms includes recurrent pyogenic Infections, thrombocytopenic Purpura, Eczema (WIPE)
- capsular polysaccharides of bacteriaに対するIgMの反応が起こせなくなる
- 緑膿菌、黄色ブドウ球菌、ヘルペスウイルス属、カンジダ属
皮膚症状=
-
合併症=
検査
- 参考1
- T細胞数の減少と機能低下。Treg細胞の機能低下 B細胞も伴うらしい。
- 免疫グロブリン:(減少~正常)IgG, IgM, (増加) IgA, IgE
- 特定のワクチン抗原に対する反応の欠如
- NK細胞数は正常~増加、細胞殺傷能力低下
- 食細胞(好中球など)の遊走能低下。
- リンパ球数:幼小児時は正常、成長につれて減少。
治療
予後
参考
- 1. [charged] Wiskott-Aldrich syndrome - uptodate [4]
国試
[★]
- 英
- mucosa-associated lymphoid tissue lymphoma MALToma
- 同
- 粘膜関連リンパ組織リンパ腫
- 関
- 胃MALTリンパ腫、悪性リンパ腫
概念
- リンパ濾胞(リンパ小節)の胚中心:{被膜側}頂部明調域(生存を許された分化したB細胞がとどまる。記憶細胞か形質細胞になってリンパ小節を去る)~基底側明調域(sIgを発現しクラススイッチを起こして中心細胞となる。中心細胞は濾胞樹状細胞から抗原提示をうける。)~暗調域(sIgを持たないB細胞(中心芽細胞)が密集。増殖盛ん)~帽状域{中心側})
- リンパ濾胞外側領域(おそらく胚中心の頂部明調領域であろう)のB細胞層にある記憶B細胞に由来する。
- 形態は小型胚中心細胞に似ており、広く明調な細胞質と切れ込みを認める。
原因
病理
- 胃MALTリンパ腫の場合、胃癌0-IIc型(表面感凹型)に似ている。
- 胃炎型の粘膜は萎縮性胃炎像、多発びらん、顆粒状、敷石状粘膜
細胞表面マーカー
特徴 YN.A-46
- 胚中心細胞に類似したcentrocyte-like cellの浸潤
- 粘膜上皮の破壊像
- 単クローン性免疫グロブリンを有する形質細胞の浸潤
- リンパ濾胞の残存
検査
染色体
- API2(11q21)、MALT1(18q21)
診断
治療
- ヘリコバクター・ピロリ菌の除菌により消失することがある
- 抗菌薬:ヘリコバクターの除菌 → 第一選択とすることが推奨されている(参考1)
- 除菌治療の有効性予測因子: H. pylori感染の有無、臨床病期、深達度、API2-MALT1 染色体転座 (参考1)
- 抗悪性腫瘍薬:(病変が広範囲に及んでいる場合 more extensive disease)chlorambucil単剤。rituximabを加えて使用するレジメンもある。(HIM.694)
- 放射線療法
- リツキシマブ
up2date
- Management of gastrointestinal lymphomas (This topic last updated: 6月 2, 2010)
- H. pylori陽性:H.pylori除菌を行う
- H. pylori陰性 or t(11;18)転座:局所放射線療法
- H. pylori陽性:H.pylori除菌を行う。免疫療法や化学療法が必要になるまで症状が進むまで経過観察。
- 消化管の切除は消化管穿孔や消化管閉塞を合併するまでreserveされる。
予後
- リンパ節転移は少なく、臨床的進行は遅い。
- 中間~良好 (LAB.1219)
参考
- 1. Helicobacter pylori 感染の診断と治療のガイドライン2009改訂版 - 日本ヘリコバクター学会
- http://www.jshr.jp/pdf/journal/guideline2009-2.pdf
- http://pathol.ncc.go.jp/matsuno/images/matsuno-43.htm
- http://user.shikoku.ne.jp/hirata/geca/sub48.html
[★]
- 英
- cluster of differentiation, CD
- Bリンパ球 CD10,CD19,CD20
- Tリンパ球 CD2,CD3,CD5,CD7
- 幹細胞 CD34
- 顆粒球 CD13,CD33
- 単球 CD14:LPSをリガンドとし、Toll-like receptorと共役して細胞内シグナルを伝達する分子。
- 巨核球 CD41(GpIIb),CD42(GpIb)
- NK細胞:CD16(IgGのFc部に対する受容体)、CD56(NCAM-I)
[★]
[★]
- 同
- T4, Leucine-SA, Leucine-3B
- 関
- 表面抗原分類