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
Beschreibung
Zusammenfassung:Copyright © 2024. Published by Elsevier Inc.
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
Beschreibung:Date Completed 14.06.2024
Date Revised 14.06.2024
published: Print-Electronic
Citation Status MEDLINE
ISSN:1521-7035
DOI:10.1016/j.clim.2024.110252