| Case |
1 |
2 |
3 |
| Fever > 38.5°C |
+ |
+ |
+ |
| Splenomegaly |
+ |
- |
+ |
| Cytopenias |
+ |
+ |
+ |
| Hypertriglyceridemia (fasting TG > 265 mg/dL) and/or hypofibrinogenemia (fibrinogen < 150 mg/dL) |
- |
+ |
+ |
| Hemophagocytosis on bone marrow biopsy |
+ |
+ |
+ |
| Elevated ferritin > 500 ng/mL |
+ |
+ |
+ |
| Low/absent NK-cell activity |
N/A |
N/A |
N/A |
| Elevated soluble CD25 > 2 standard deviations above normal range |
+ |
+ |
+ |
| Presented with hyponatremia |
+ |
+ |
+ |
| Gastrointestinal/biliary inflammatory changes on imaging |
+ |
+ |
+ |
