Table 1.
Baseline demographic and clinical characteristics of patients with HLH.
| Total | LAHS | Benign disease-associated HLH | |
|---|---|---|---|
| n = 74 | n = 41 | n = 33 | |
| Age, yr | 39.0 (22.3–54.3) | 44.0 (24.5–54.0) | 31.0 (19.0–55.0) |
| Male, number (%) | 44 (59.5%) | 28 (68.3%) | 16 (48.5%) |
| Fever (>38°C) | 73 (98.6%) | 41 (100%) | 32 (97.0%) |
| Splenomegaly | 63 (85.1%) | 36 (87.8%) | 27 (81.8%) |
| Leukocytes, ×109/L | 2.7 (1.2–4.0) | 2.6 (1.1–3.7) | 3.2 (1.5–4.5) |
| Hemoglobin, g/L | 86.0 (76.0–103.8) | 86.0 (72.5–105.0) | 86.0 (77.0–102.0) |
| Platelets, ×109/L | 36.9 (25.0–66.3) | 36.7 (21.0–83.0) | 39.0 (25.9–56.0) |
| Triglycerides, mmol/L | 2.5 (1.6–3.6) | 2.7 (2.1–3.8) | 2.1 (1.5–3.5) |
| Fibrinogen, g/L | 1.5 (0.9–2.6) | 1.3 (0.9–2.6) | 1.7 (1.1–2.6) |
| Ferritin, μg/L | 6268.5 (1975.0–14802.5) | 6500.0 (1825.5–15000) | 5973.0 (2023.5–14734.5) |
| NK cell activity (%) | 12.4 (9.6–16.3) | 11.7 (8.4–17.1) | 12.7 (11.4–16.0) |
| sCD25 ≥ 2400 U/mL | 70 (94.6%) | 40 (97.6%) | 30 (90.9%) |
| Hemophagocytosis | 61 (82.4%) | 32 (78.0%) | 29 (87.9%) |
Results reported as median (range) or number (%).