Clinical and laboratory manifestations of HLH in presented cases as classified according to HLH-2004 diagnostic criteria and the HScore
| Classification . | Case 1 . | Case 2 . |
|---|---|---|
| HLH-2004 diagnostic criteria5 | ||
| Fevers | Yes (Tmax 38.9°C) | Yes (Tmax 39.5°C at first hospital) |
| Splenomegaly | Yes | Yes |
| Peripheral blood cytopenias (>2 lineages)* | Yes (platelet nadir 84 × 109/L, hemoglobin nadir 2.8 g/dL) | Yes (platelet nadir 85 × 109/L, hemoglobin nadir 8.5 g/dL) |
| Hypertriglyceridemia (>265 mg/dL) | Yes (269 mg/dL) | Yes (369 mg/dL) |
| Hemophagocytosis | Yes | Yes |
| Hyperferritinemia | Yes (58 505 ng/mL) | Yes (24 919 ng/mL) |
| Low/absent NK cell activity | No | No |
| Elevated soluble CD25 | Not assessed | Not assessed |
| HLH-associated mutations | No | No |
| HScore14,15 | ||
| Known immunosuppression | No (+0 pts) | No (+0 pts) |
| Temperature | 38.4-39.4 (+33 pts) | >39.4 (+49 pts) |
| Organomegaly | Splenomegaly (+23 pts) | Splenomegaly (+23 pts) |
| Lineages of cytopenias† | 2 (+24 pts) | 2 (+24 pts) |
| Ferritin, ng/mL | >6000 (+50 pts) | >6000 (+50 pts) |
| Triglyceride, mg/dL | 132.7-354 (+44 pts) | >354 (+64 pts) |
| Fibrinogen, mg/dL | >250 (+0 pts) | >250 (+0 pts) |
| AST | ≥30 (+19 pts) | ≥30 (+19 pts) |
| Hemophagocytosis on bone marrow | Yes (+35 pts) | Yes (+35 pts) |
| Total HScore | 228 (96%-98% probability of HLH) | 264 (>99% probability of HLH) |
| Classification . | Case 1 . | Case 2 . |
|---|---|---|
| HLH-2004 diagnostic criteria5 | ||
| Fevers | Yes (Tmax 38.9°C) | Yes (Tmax 39.5°C at first hospital) |
| Splenomegaly | Yes | Yes |
| Peripheral blood cytopenias (>2 lineages)* | Yes (platelet nadir 84 × 109/L, hemoglobin nadir 2.8 g/dL) | Yes (platelet nadir 85 × 109/L, hemoglobin nadir 8.5 g/dL) |
| Hypertriglyceridemia (>265 mg/dL) | Yes (269 mg/dL) | Yes (369 mg/dL) |
| Hemophagocytosis | Yes | Yes |
| Hyperferritinemia | Yes (58 505 ng/mL) | Yes (24 919 ng/mL) |
| Low/absent NK cell activity | No | No |
| Elevated soluble CD25 | Not assessed | Not assessed |
| HLH-associated mutations | No | No |
| HScore14,15 | ||
| Known immunosuppression | No (+0 pts) | No (+0 pts) |
| Temperature | 38.4-39.4 (+33 pts) | >39.4 (+49 pts) |
| Organomegaly | Splenomegaly (+23 pts) | Splenomegaly (+23 pts) |
| Lineages of cytopenias† | 2 (+24 pts) | 2 (+24 pts) |
| Ferritin, ng/mL | >6000 (+50 pts) | >6000 (+50 pts) |
| Triglyceride, mg/dL | 132.7-354 (+44 pts) | >354 (+64 pts) |
| Fibrinogen, mg/dL | >250 (+0 pts) | >250 (+0 pts) |
| AST | ≥30 (+19 pts) | ≥30 (+19 pts) |
| Hemophagocytosis on bone marrow | Yes (+35 pts) | Yes (+35 pts) |
| Total HScore | 228 (96%-98% probability of HLH) | 264 (>99% probability of HLH) |