About: Primary and secondary hemophagocytic lymphohistiocytosis have different patterns of T‐cell activation, differentiation and repertoire

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About: Primary and secondary hemophagocytic lymphohistiocytosis have different patterns of T‐cell activation, differentiation and repertoire Goto Sponge NotDistinct Permalink

An Entity of Type : schema:ScholarlyArticle, within Data Space : covidontheweb.inria.fr associated with source document(s)

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type	Academic Article research paper schema:ScholarlyArticle
isDefinedBy	Covid-on-the-Web dataset
has title	Primary and secondary hemophagocytic lymphohistiocytosis have different patterns of T‐cell activation, differentiation and repertoire
Creator	Ehl, Stephan Ammann, Sandra Article, Research Bode, Sebastian Fuchs, Ilka Heeg, Maximilian Janka, Gritta Klemann, Christian Lehmberg, Kai Zur Stadt, Udo Rensing-Ehl, Anne
Source	PMC
abstract	Hemophagocytic lymphohistiocytosis (HLH) is a life‐threatening inflammatory syndrome characterized by hyperactivation of lymphocytes and histiocytes. T cells play a key role in HLH pathogenesis, but their differentiation pattern is not well characterized in patients with active HLH. We compared T‐cell activation patterns between patients with familial HLH (1°HLH), 2°HLH without apparent infectious trigger (2°HLH) and 2°HLH induced by a viral infection (2°V‐HLH). Polyclonal CD8(+) T cells are highly activated in 1°HLH and 2°V‐HLH, but less in 2°HLH as assessed by HLA‐DR expression and marker combination with CD45RA, CCR7, CD127, PD‐1 and CD57. Absence of increased HLA‐DR expression on T cells excluded active 1° HLH with high sensitivity and specificity. A high proportion of polyclonal CD127(−)CD4(+) T cells expressing HLA‐DR, CD57, and perforin is a signature of infants with 1°HLH, much less prominent in virus‐associated 2°HLH. The similar pattern and extent of CD8(+) T‐cell activation compared to 2° V‐HLH is compatible with a viral trigger of 1°HLH. However, in most 1°HLH patients no triggering infection was documented and the unique activation of cytotoxic CD4(+) T cells indicates that the overall T‐cell response in 1°HLH is different. This may reflect different pathways of pathogenesis of these two HLH variants.
has issue date	2017-01-03(xsd:dateTime)
bibo:doi	10.1002/eji.201646686
bibo:pmid	27925643
has license	no-cc
sha1sum (hex)	2774b6c2b3acf470569fd6b372f5b4edb006a811
schema:url	https://doi.org/10.1002/eji.201646686
resource representing a document's title	Primary and secondary hemophagocytic lymphohistiocytosis have different patterns of T‐cell activation, differentiation and repertoire
has PubMed Central identifier	PMC7163530
has PubMed identifier	27925643
schema:publication	Eur J Immunol
resource representing a document's body	covid:2774b6c2b3acf470569fd6b372f5b4edb006a811#body_text
is schema:about of	named entity 'Hemophagocytic lymphohistiocytosis' named entity 'HLA-DR' named entity 'MAY BE' named entity 'PERFORIN' named entity 'PRIMARY' named entity 'activation' named entity 'gene' named entity 'University of Freiburg' named entity 'leishmania' named entity 'HHV-6' named entity 'blood cultures' named entity 'infection' named entity 'T-cell activation' named entity 'MHC class II' named entity 'treatment of patients' named entity 'HLA-DR' named entity 'immune cells' named entity 'Adenovirus' named entity 'HLH' named entity 'liver' named entity 'HLH' named entity 'microbial' named entity 'CD8 +' named entity 'sCD25' named entity 'autoinflammatory disease' named entity 'perforin' named entity 'infection' named entity 'perforin' named entity 'autoimmune' named entity 'family history' named entity 'Oncology' named entity 'chemoluminescence' named entity 'IL-7 receptor' named entity 'PBMCs' named entity 'histiocytosis' named entity 'HLA-DR' named entity 'infection' named entity 'pneumonia' named entity '12,000' named entity 'HIV' named entity 'MHC' named entity 'CD4 +' named entity 'immunodeficient' named entity 'sensitivity and specificity' named entity 'perforin' named entity 'genetic predisposition' named entity 'HLA-DR' named entity 'inflammatory response' named entity 'FHL' named entity 'CD57' named entity 'T-cell activation' named entity 'HLH' named entity 'T-cell activation' named entity 'HLH' named entity 'CD27' named entity 'diagnostic criteria' named entity 'ATG' named entity 'CD28' named entity 'STX11' named entity 'MAS' named entity 'CD8 +' named entity 'HLH' named entity 'perforin' named entity 'HLH' named entity 'T-cell activation' named entity 'Hemophagocytic lymphohistiocytosis' named entity 'Perforin' named entity 'T-cell activation' named entity 'CD4 +' named entity 'T-cell' named entity 'CD4 +'

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