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.jpg): Failed to open stream: No such file or directory in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 117 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
PMID27925643
show 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°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.