Serum proteomics reveals hemophagocytic lymphohistiocytosis-like phenotype in a subset of patients with multisystem inflammatory syndrome in children

Serum proteomics reveals hemophagocytic lymphohistiocytosis-like phenotype in a subset of patients with multisystem inflammatory syndrome in children

Copyright © 2024. Published by Elsevier Inc.

Bibliographische Detailangaben
Veröffentlicht in:Clinical immunology (Orlando, Fla.). - 1999. - 264(2024) vom: 01. Juni, Seite 110252
1. Verfasser: Tulling, Adam J (VerfasserIn)
Weitere Verfasser: Holierhoek, Marloes G, Jansen-Hoogendijk, Anja M, Hoste, Levi, Haerynck, Filomeen, Tavernier, Simon J, Oostenbrink, Rianne, Buysse, Corinne M P, Bannier, Michiel A G E, Bekhof, Jolita, Breukels, Mijke, Hammer, Sanne C, Jacobs, Monique A M, Kamps, Arvid W A, van der Linden, Jan W, Lebon, Ankie, Oudshoorn, Johanna H, Tramper-Stranders, Gerdien A, Vastert, Sebastiaan J, Wieringa, Jantien W, Terheggen-Lagro, Suzanne W J, Wildenbeest, Joanne G, von Asmuth, Erik G J, van den Akker, Erik B, van Gijn, Marielle E, Lugthart, Gertjan, Buddingh, Emilie P
Format: Online-Aufsatz
Sprache:English
Veröffentlicht: 2024
Zugriff auf das übergeordnete Werk:Clinical immunology (Orlando, Fla.)
Schlagworte:Journal Article COVID-19 HLH Immune dysregulation MIS-C SARS-CoV-2
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520 |a Children with Multisystem Inflammatory Syndrome in Children (MIS-C) can present with thrombocytopenia, which is a key feature of hemophagocytic lymphohistiocytosis (HLH). We hypothesized that thrombocytopenic MIS-C patients have more features of HLH. Clinical characteristics and routine laboratory parameters were collected from 228 MIS-C patients, of whom 85 (37%) were thrombocytopenic. Thrombocytopenic patients had increased ferritin levels; reduced leukocyte subsets; and elevated levels of ASAT and ALAT. Soluble IL-2RA was higher in thrombocytopenic children than in non-thrombocytopenic children. T-cell activation, TNF-alpha and IFN-gamma signaling markers were inversely correlated with thrombocyte levels, consistent with a more pronounced cytokine storm syndrome. Thrombocytopenia was not associated with severity of MIS-C and no pathogenic variants were identified in HLH-related genes. This suggests that thrombocytopenia in MIS-C is not a feature of a more severe disease phenotype, but the consequence of a distinct hyperinflammatory immunopathological process in a subset of children 
650 4 |a Journal Article 
650 4 |a COVID-19 
650 4 |a HLH 
650 4 |a Immune dysregulation 
650 4 |a MIS-C 
650 4 |a SARS-CoV-2 
700 1 |a Holierhoek, Marloes G  |e verfasserin  |4 aut 
700 1 |a Jansen-Hoogendijk, Anja M  |e verfasserin  |4 aut 
700 1 |a Hoste, Levi  |e verfasserin  |4 aut 
700 1 |a Haerynck, Filomeen  |e verfasserin  |4 aut 
700 1 |a Tavernier, Simon J  |e verfasserin  |4 aut 
700 1 |a Oostenbrink, Rianne  |e verfasserin  |4 aut 
700 1 |a Buysse, Corinne M P  |e verfasserin  |4 aut 
700 1 |a Bannier, Michiel A G E  |e verfasserin  |4 aut 
700 1 |a Bekhof, Jolita  |e verfasserin  |4 aut 
700 1 |a Breukels, Mijke  |e verfasserin  |4 aut 
700 1 |a Hammer, Sanne C  |e verfasserin  |4 aut 
700 1 |a Jacobs, Monique A M  |e verfasserin  |4 aut 
700 1 |a Kamps, Arvid W A  |e verfasserin  |4 aut 
700 1 |a van der Linden, Jan W  |e verfasserin  |4 aut 
700 1 |a Lebon, Ankie  |e verfasserin  |4 aut 
700 1 |a Oudshoorn, Johanna H  |e verfasserin  |4 aut 
700 1 |a Tramper-Stranders, Gerdien A  |e verfasserin  |4 aut 
700 1 |a Vastert, Sebastiaan J  |e verfasserin  |4 aut 
700 1 |a Wieringa, Jantien W  |e verfasserin  |4 aut 
700 1 |a Terheggen-Lagro, Suzanne W J  |e verfasserin  |4 aut 
700 1 |a Wildenbeest, Joanne G  |e verfasserin  |4 aut 
700 1 |a von Asmuth, Erik G J  |e verfasserin  |4 aut 
700 1 |a van den Akker, Erik B  |e verfasserin  |4 aut 
700 1 |a van Gijn, Marielle E  |e verfasserin  |4 aut 
700 1 |a Lugthart, Gertjan  |e verfasserin  |4 aut 
700 1 |a Buddingh, Emilie P  |e verfasserin  |4 aut 
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