%0 Journal Article
%T Serum proteomics reveals hemophagocytic lymphohistiocytosis-like phenotype in a subset of patients with multisystem inflammatory syndrome in children.
%A Tulling AJ
%A Holierhoek MG
%A Jansen-Hoogendijk AM
%A Hoste L
%A Haerynck F
%A Tavernier SJ
%A Oostenbrink R
%A Buysse CMP
%A Bannier MAGE
%A Bekhof J
%A Breukels M
%A Hammer SC
%A Jacobs MAM
%A Kamps AWA
%A van der Linden JW
%A Lebon A
%A Oudshoorn JH
%A Tramper-Stranders GA
%A Vastert SJ
%A Wieringa JW
%A Terheggen-Lagro SWJ
%A Wildenbeest JG
%A von Asmuth EGJ
%A van den Akker EB
%A van Gijn ME
%A Lugthart G
%A Buddingh EP
%J Clin Immunol
%V 264
%N 0
%D 2024 Jul 12
%M 38744408
%F 10.19
%R 10.1016/j.clim.2024.110252
%X 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.