Table 2

Differential diagnosis of CRS6

Familial HLHSecondary HLH/MASCRS related to HLH or MASSepsis
Genetics Homozygous mutationHeterozygous mutations in case-reportsNot reportedNot reported
Biomarkers
IL-10 Very highVery highHighHigh
IFN-alpha Very highVery highVery highNormal
IL-6 High High Very high Very high
Ferritin Very highVery highVery highHigh
CD 163 Very highVery highHighNot reported
  • CRS, cytokine release syndrome; HLH, hemophagocytic lymphohistiocytosis; IFN, interferon; IL-6, interleukin-6; MAS, macrophage activation syndrome.