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Table 2 Differential diagnoses of CRS-related HLH/MAS

From: Cytokine release syndrome

  Familial HLH Secondary HLH/MAS CRS-related HLH/MAS Sepsis
Genetic Predisposition Homozygous mutations Heterozygous mutations in some patients unknown unknown
Age group Young children All ages All ages All ages
Biomarkers
 IL-10 ↑↑↑ ↑↑↑
 IFN-γ ↑↑↑ ↑↑↑ ↑↑↑ ←→
 IL-6 ↑↑↑ ↑↑↑
 Ferritin ↑↑↑ ↑↑↑ ↑↑↑
 CD163 ↑↑↑ ↑↑↑ NDA
  1. CRS cytokine release syndrome, HLH hemophagocytic lymphohistiocytosis, MAS macrophage activation syndrome, Sepsis. NDA no data available