Table 2.
Analysis of HLH diagnosis parameters in various HLH-prone mouse models on day 12 after LCMV infection.
| HLH parameter | Wild-type | Beige | Pearl | Souris | Syntaxin-11KO | Ashen | PKO |
|---|---|---|---|---|---|---|---|
| Fever/hypothermia | 37.0 ± 0.4 | 37.0 ± 0.4 | 37.0 ± 0.4 | 35.4 ± 0.8* | 34.7 ± 0.7* | 34.1 ± 0.3* | 34.4 ± 0.9* |
| WBC (×103/μL) | 10.3 ± 3.4 | 14.3 ± 7.0 | 13.7 ± 5.5 | 6.9 ± 3.5* | 3.84 ± 2.1* | 6.1 ± 3.5* | 4.1 ± 2.0* |
| HGB (g/dL) | 11.3 ± 1.5 | 10.6 ± 1.2 | 10.7 ± 1.2 | 6.5 ± 1.4*** | 3.8 ± 0.6*** | 3.0 ± 0.6*** | 4.6 ± 1.0*** |
| PLT (×103/μL) | 839 ± 180 | 803 ± 213 | 441 ± 11*** | 253 ± 75*** | 507.1 ± 156.5*** | 221 ± 151*** | 228 ± 114*** |
| Ferritin (ng/mL) | 525.2 ± 199.4 | 508.6 ± 105.7 | 817.7 ± 296.3 | 1009.0 ± 158.2 | 1624.3 ± 761.9*** | 3202.0 ± 568.5** | 2951.1 ± 2098.6*** |
| sCD25 (pg/mL) | 271.9 ± 121.6 | 291.1 ± 46.0 | 321.3 ± 195.3 | 631.7 ± 166.4*** | 709.1 ± 98.3*** | 833.3 ± 238.6*** | 823.5 ± 105.2*** |
| Triglycerides (mg/dL) | 71.1 ± 18.7 | 75.3 ± 26.2 | 88.7 ± 12.3 | 82.5 ± 35.4 | 216.0 ± 69.0*** | 146.7 ± 37.8 | 111.4 ± 62.0 |
| Hemophagocytosis (liver) | (+) | + | ++ | +++ | +++ | +++ | +++ |
| Splenomegaly | + | + | + | + | + | + | + |
| NK cell cytotoxicity | Normal | ↓↓ | ↓ | ↓↓↓ | ↓↓ | ↓↓↓ | ↓↓↓ |
| Additional parameter | |||||||
| LDH (U/L) | 502.7 ± 171.7 | 493.3 ± 161.2 | 1379.0 ± 1231.2 | 1464.7 ± 422.5* | 978.0 ± 692.8 | 2053.3 ± 871.8* | 1944.2 ± 1535.7** |
| GLDH (U/L) | 11.1 ± 3.7 | 15.8 ± 6.8 | 121.9 ± 293.76* | 181.6 ± 136.6*** | 113.5 ± 98.9*** | 345.9 ± 93.9*** | 156.1 ± 103.1*** |
| IFN-γ (ng/mL) | b.d. | b.d. | b.d. | 23.6 ± 6.2*** | 23.5 ± 12.9*** | 38.0 ± 12.5*** | 26.2 ± 16.7*** |
| HLH | NO | NO | Transient | YES | YES | YES | Lethal |
Red fields indicate fulfilled HLH criteria.
The red font accentuates the final disease phenotype.
***p < 0.001; **p < 0.01; *p < 0.05 (ANOVA; statistically significant differences compared to day 12 values of wild-type mice).
b.d. below detection limit.