Murine models of secondary hemophagocytic lymphohistiocytosis (HLH) are compared with the prototypical model of primary HLH (prf−/− mice infected with LCMV).
| Primary | EBV HM | Secondary | IL-6TG | ||
|---|---|---|---|---|---|
| LCMV in PRF1 −/− | Salmonella enterica | Repeated CpG Dinucleotide | |||
| Genetics of cytotoxic pathway | Perforin −/− | Normal | Normal | Normal | Normal |
| Trigger | LCMV | EBV | S. enterica | CpG | LPS/Poly:IC |
| Background inflammation | No | No | No | No | Yes |
| Lethality | Yes 2 weeks | Yes 10 weeks | No | No | Yes one week |
| Splenomegaly | Yes | Yes | Yes | Yes | Yes* |
| Thrombocytopenia | Yes | Yes | Yes | Yes | Yes |
| Leukocytes | Neutropenia | Lymphocytosis | Leukocytosis | Lymphopenia | Neutropenia |
| Anemia | Yes | Yes | Yes | Yes | Yes |
| Ferritin increase | Yes** | Not reported | Yes | Yes | Yes |
| LDH increase | Yes*** | Not reported | Not reported | Not reported | Yes |
| Fibrinogen | Decreased | Not reported | Mild decrease | Normal | Not reported |
| IL-6 | Increased | Not reported | Not reported | Increased | Increased |
| IL-1ß | Not detected | Not reported | Not reported | Not reported | Increased |
| IFN-γ | Increased | Increased | Not reported | Increased | Increased* |
| Hemophagocytosis | Yes | Yes | Yes | No | No |
| Rescue upon T CD8+ depletion | Yes | Not reported | Not reported | No | Not reported |
| Rescue upon IFN-γ blockade | Yes | Not reported | Not reported | Yes | Not reported |