Abstract:
:Autoimmune lymphoproliferative syndrome (ALPS) is characterized by immune dysregulation due to a defect in lymphocyte apoptosis. The clinical manifestations may be noted in multiple family members and include lymphadenopathy, splenomegaly, increased risk of lymphoma, and autoimmune disease, which typically involves hematopoietic cell lines manifesting as multilineage cytopenias. Since the disease was first characterized in the early 1990s, there have been many advances in the diagnosis and management of this syndrome. The inherited genetic defect of many ALPS patients has involved (FAS) pathway signaling proteins, but there remain those patients who carry undefined genetic defects. Despite ALPS having historically been considered a primary immune defect presenting in early childhood, adult onset presentation is increasingly becoming recognized and more so in genetically undefined patients and those with somatic FAS mutations. Thus, future research may identify novel pathways and/or regulatory proteins important in lymphocyte activation and apoptosis.
journal_name
Curr Allergy Asthma Repjournal_title
Current allergy and asthma reportsauthors
Shah S,Wu E,Rao VK,Tarrant TKdoi
10.1007/s11882-014-0462-4subject
Has Abstractpub_date
2014-09-01 00:00:00pages
462issue
9eissn
1529-7322issn
1534-6315journal_volume
14pub_type
杂志文章,评审abstract::Specific allergen immunotherapy (SIT) is the only disease-modifying treatment for allergic rhinitis and asthma. Subcutaneous immunotherapy (SCIT) is the only method with a US Food and Drug Administration (FDA)-approved formulation, but safety concerns limit administration to medical facilities. Sublingual immunotherap...
journal_title:Current allergy and asthma reports
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journal_title:Current allergy and asthma reports
pub_type: 杂志文章,评审
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journal_title:Current allergy and asthma reports
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更新日期:2010-05-01 00:00:00
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pub_type: 杂志文章,评审
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journal_title:Current allergy and asthma reports
pub_type: 杂志文章,评审
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更新日期:2015-08-01 00:00:00
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journal_title:Current allergy and asthma reports
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更新日期:2004-07-01 00:00:00
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journal_title:Current allergy and asthma reports
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更新日期:2006-05-01 00:00:00
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journal_title:Current allergy and asthma reports
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更新日期:2020-01-16 00:00:00
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pub_type: 杂志文章,评审
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更新日期:2003-01-01 00:00:00
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更新日期:2013-12-01 00:00:00
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journal_title:Current allergy and asthma reports
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更新日期:2001-11-01 00:00:00
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更新日期:2002-01-01 00:00:00
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更新日期:2003-11-01 00:00:00
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更新日期:2001-09-01 00:00:00
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journal_title:Current allergy and asthma reports
pub_type: 杂志文章,评审
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更新日期:2009-11-01 00:00:00
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journal_title:Current allergy and asthma reports
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更新日期:2011-02-01 00:00:00
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更新日期:2020-06-06 00:00:00