Overview of clinical features included in revised diagnostic guideline of HLH within case group and control group.
| Clinical Features | Case Group, n = 18 | Control Group, n = 72 |
|---|---|---|
| A molecular diagnosis consistent with HLH | NA | NA |
| Fever (≥ 38.5°C for ≥ 7 days) | 18 (100.0) | 11 (15.3) |
| Splenomegaly | 13 (72.2) | 6 (8.3) |
| Cytopenias aYecting 2 of 3 lineages in the peripheral blood | 18 (100.0) | 0 (0.0) |
| Hemoglobin < 90 g/l | 17 (94.4) | 5 (6.9) |
| Platelets < 100 × 109/l | 18 (100.0) | 8 (11.1) |
| Neutrophils < 1.0 × 109/l | 5 (27.8) | 1 (1.4) |
| Hypertriglyceridemia and/or hypo[brinogenemia | 18 (100.0) | 14 (19.4) |
| Fasting triglycerides ≥ 3.0 mmol/l (i.e., 265 mg/dl) | 10 (55.6) | 11 (15.3) |
| Fibrinogen ≤ 1.5 g/l | 12 (66.7) | 3 (4.2) |
| Hemophagocytosis in bone marrow | 9 (50.0) | 0 (0.0) |
| Low or absent NK cell activity | NA | NA |
| Ferritin ≥ 500 mg/l | 18 (100.0) | 40 (55.6) |
| Soluble CD25 (i.e., soluble IL-2 receptor) ≥ 2400 u/ml | NA | NA |
Data are n (%). HLH: Hemophagocytic lymphohistiocytosis; NK cell: natural killer cell; IL-2: interleukin 2; NA: not applicable.