Table 2 Differential diagnoses of CRS-related HLH/MAS
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 | ↑ |