Serum proteomics reveals hemophagocytic lymphohistiocytosis-like phenotype in a subset of patients with multisystem inflammatory syndrome in children
Tulling, Adam J; 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
(2024) Clinical Immunology, volume 264
(Article)
Abstract
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.
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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.
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Keywords: COVID-19, HLH, Immune dysregulation, MIS-C, SARS-CoV-2, Immunology and Allergy, Immunology, Journal Article
ISSN: 1521-6616
Publisher: Academic Press Inc.
Note: Publisher Copyright: © 2024
(Peer reviewed)