Table 1 Clinical features, underlying diseases, and therapy of the two groups of patients
| Â | Group R number [percentage (%)] | Group C number [percentage (%)] | |
|---|---|---|---|
Clinical features | Fever ≥38.5 °C | 11 (100) | 11 (100) |
Splenomegaly | 11 (100) | 11 (100) | |
Cytopenias | 11 (100) | 10 (90.91) | |
Hypofibrinogenemia/ Hypertriglyceridemia | 11 (100) | 10 (90.91) | |
Elevated ferritin | 11 (100) | 11 (100) | |
Hemophagocytosis | 7 (63.64) | 7 (63.64) | |
Low or absent NK-cell activity | 4 (36.36) | 7 (63.64) | |
Elevated soluble CD25 | 7 (63.64) | 7 (63.64) | |
Underlying diseases | Infection | 8 (72.73) | 4 (36.36) |
Juvenile idiopathic arthritis (systemic type) | 2 (18.18) | 6 (54.55) | |
Kawasaki disease | 1 (9.10) | / | |
Systemic lupus erythematosus | / | 1 (9.10) | |
Therapy (Group R: before ruxolitinib) | HLH-04 MP and IVIG | 4 (36.36) 3 (27.27) | 6 (54.55) 5 (45.45) |
IVIG | 4 (36.36) | / | |