Table 3 Clinical information on 45 HLH patients

 ≤12 months

9/45

2/4

7/41

0.116

 >12 months

36/45

2/4

34/41

 Mean age (year, range)

3.7 (0–12.3)

2.9 (1.0–6.3)

3.8 (0–12.3)

0.646

Sex (M/F)

27/18

3/1

24/17

0.521

Fever

45/45

4/4

41/41

Hemoglobin (<90 g/L)

16/45

1/4

15/41

0.644

 Mean (g/L, range)

94.0 (46.0–135.0)

93.8 (84.0–102.0)

94.0 (46.0–135.0)

0.905

Platelets (<100x10⁹/L)

30/45

2/4

28/41

0.459

 Mean (100x10⁹/L, range)

82.5 (32.0–400.0)

100.0 (5.0–215.0)

80.8 (3.0–400.0)

0.661

 Mean (1x10⁹/L, range)

7.5 (0.3–41.3)

6.5 (1.0–9.7)

7.6 (0.3–41.3)

0.842

 Mean (%, range)

38.2 (2.0–81.5)

12.8 (6.9–19.4)

40.7 (2.0–81.5)

0.012

Total neutrophils (<1x10⁹/L)

20/45

2/4

18/41

0.815

 Mean (1x10⁹/L, range)

2.9 (0.1–22.1)

0.9 (0.1–1.3)

3.1 (0.1–22.1)

0.413

 Mean (%, range)

50.4 (7.9–88)

75.3 (71.8–77.7)

48.0 (7.9–88.0)

0.012

 Mean (1x10⁹/L, range)

3.6 (0.2–28.1)

4.9 (0.7–7.5)

3.4 (0.2–28.1)

0.175

Triglycerides (>3.0 mmol/L)

15/45

3/4

12/41

0.064

 Mean (mmol/L, range)

2.7 (0.8–8.9)

3.0 (1.3–4.1)

2.7 (0.8–8.9)

0.425

Fibrinogen (<1.5 g/L)

30/45

4/4

26/41

0.138

 Mean (g/L, range)

1.5 (0.2–4.9)

0.9 (0.5–1.2)

1.5 (0.2–4.9)

0.14

LDH (>500 IU/L)

32/45

3/4

29/41

0.857

 Mean (IU/L, range)

1018.0 (167.0–6565.0)

814.0 (363.0–1644.0)

1037.9 (167.0–6565.0)

0.842

Ferritin (>500 μg/L)

45/45

4/4

41/41

 Mean (μg/L, range)

1477.7 (895.0–1500.0)

1500.0 (1500.0–1500.0)

1474.2 (895.0–1500.0)

0.655

sIL2R (≥2400 U/ml)

26/27

4/4

22/23

0.671

 Mean (U/ml, range)

34092.7 (155.6–85787.7)

27198.1 (14250.9–38203.1)

35239.6 (155.6–85787.7)

0.922

 Mean (U/pg, range)

23.0 (0.1–57.2)

18.4 (9.5–25.5)

23.8 (0.1–57.2)

0.811

Degranulation (CD107a <5 %)

16/36

1/3

15/33

0.795

 Mean (%, range)

10.3 (0.1–35.1)

22.3 (0.7–33.8)

9.2 (0.1–35.1)

0.407

EBV-DNA (>1000 copies)

26/45

2/4

24/41

0.741

CMV-DNA (>1000 copies)

1/45

0/4

1/41

0.752