| Autoimmunity, autoinflammation and lymphoma in combined immunodeficiency (CID). | |
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MedLine Citation:
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PMID: 20146941 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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A number of primary immunodeficiencies are associated with autoimmune phenomena, e.g. Wiskott-Aldrich Syndrome, Common Variable Immunodeficiency and Hyper-IgM Syndrome. The common denominator is a dysregulation of immune responses affecting T and B cells with central and/or peripheral tolerance mechanisms being disturbed. Autoimmunity and autoinflammation may also occur in atypical phenotypes of combined immunodeficiencies (CID) usually associated with severe infectious complications. These unexpected presentations of classical CID are very instructive in how low numbers of T and B cells go hand in hand with skewing of lymphoid repertoires and function. The resulting immune dysregulation may lead to self-reactivity with organ damage and malignancy. |
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Authors:
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Catharina Schuetz; Tim Niehues; Wilhelm Friedrich; Klaus Schwarz |
Publication Detail:
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Type: Journal Article; Review Date: 2010-02-08 |
Journal Detail:
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Title: Autoimmunity reviews Volume: 9 ISSN: 1873-0183 ISO Abbreviation: Autoimmun Rev Publication Date: 2010 May |
Date Detail:
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Created Date: 2010-05-11 Completed Date: 2010-09-20 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 101128967 Medline TA: Autoimmun Rev Country: Netherlands |
Other Details:
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Languages: eng Pagination: 477-82 Citation Subset: IM |
Copyright Information:
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2010 Elsevier B.V. All rights reserved. |
Affiliation:
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Department of Pediatrics and Adolescent Medicine, University Hospital Ulm, Germany. catharina.schuetz@uniklinik-ulm.de |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Animals Autoantigens / immunology* Autoimmunity B-Lymphocytes / immunology Humans Immune Tolerance Inflammation Lymphoma / complications, immunology* Severe Combined Immunodeficiency / complications, immunology* T-Lymphocytes / immunology |
| Chemical | |
Reg. No./Substance:
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0/Autoantigens |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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