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10.1002/eji.201646686

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suck abstract from ncbi


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pmid27925643      Eur+J+Immunol 2017 ; 47 (2): 364-373
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  • Primary and secondary hemophagocytic lymphohistiocytosis have different patterns of T-cell activation, differentiation and repertoire #MMPMID27925643
  • Ammann S; Lehmberg K; Zur Stadt U; Janka G; Rensing-Ehl A; Klemann C; Heeg M; Bode S; Fuchs I; Ehl S
  • Eur J Immunol 2017[Feb]; 47 (2): 364-373 PMID27925643show ga
  • 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 degrees HLH), 2 degrees HLH without apparent infectious trigger (2 degrees HLH) and 2 degrees HLH induced by a viral infection (2 degrees V-HLH). Polyclonal CD8(+) T cells are highly activated in 1 degrees HLH and 2 degrees V-HLH, but less in 2 degrees 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 degrees 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 degrees HLH, much less prominent in virus-associated 2 degrees HLH. The similar pattern and extent of CD8(+) T-cell activation compared to 2 degrees V-HLH is compatible with a viral trigger of 1 degrees HLH. However, in most 1 degrees 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 degrees HLH is different. This may reflect different pathways of pathogenesis of these two HLH variants.
  • |CD57 Antigens/metabolism[MESH]
  • |CD8-Positive T-Lymphocytes/*immunology[MESH]
  • |Cell Differentiation[MESH]
  • |Cells, Cultured[MESH]
  • |Female[MESH]
  • |HLA-DR Antigens/metabolism[MESH]
  • |Humans[MESH]
  • |Infant[MESH]
  • |Infant, Newborn[MESH]
  • |Lymphocyte Activation[MESH]
  • |Lymphohistiocytosis, Hemophagocytic/diagnosis/etiology/*immunology[MESH]
  • |Male[MESH]
  • |Perforin/metabolism[MESH]
  • |Receptors, Antigen, T-Cell/genetics[MESH]


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