Supplementary Table I.
Clinical features at presentation of the patients with sHLH studied
| Patients, N | 14 |
|---|---|
| Fever, n/N (%) | 12/14 (85.7) |
| Splenomegaly, n/N (%) | 5/14 (35.7) |
| Hepatomegaly, n/N (%) | 7/14 (50) |
| CNS involvement, n/N (%) | 4/14 (28.6) |
| Haemorrhages, n/N (%) | 2/14 (14.3) |
| Anemia (<90 g/L), n/N (%) | 8/14 (57.1) |
| Trombocytopenia (<100 × 109/L), n/N (%) | 5/14 (35.7) |
| Leukopenia (<4 × 109/L), n/N (%) | 5/14 (35.7) |
| Neutropenia (<1 × 109/L), n/N (%) | 3/14 (21.4) |
| Hypertriglyceridemia (≥265 mg/dl), n/N (%) | 10/14 (71.4) |
| Hypofibrinogenemia (≤1.5 g/l), n/N (%) | 6/14 (42.9) |
| Hemophagocytosis, n/N (%) | 12/14 (85.7) |
| Ferritin (≥500 mcg/L), n/N (%) | 13/14 (92.8) |
Abbreviation: sHLH, secondary hemophagocytic lymphohistiocytosis.
14 patients (age at onset 8.6 years, interquartile range (IQR) 4.1–12.9 years; female 36%) met the 2004-HLH diagnostic guidelines: 7 patients met 5 and 7 patients met 4 criteria. The table shows the number and the proportion of patients who developed each of the diagnostic criteria.