Table 2 HLH-associated laboratory parameters in LCH patients with MAS-HLH

Whole blood counts

 Hemoglobin (g/L)

110–160

70.0 (49.0–99.0)

  90–70

–

15 (53.6%)

  < 70

–

13 (46.4%)

 Platelets (× 10⁹/L)

100–400

49.5 (1.0–422.0)

  ≥ 100

–

3 (10.7%)

  < 100

–

25 (89.3%)

 Neutrocytes (× 10⁹/L)

0.7–4.6

2.5 (0.7–17.7)

  ≥ 1.0

–

19 (67.9%)

  < 1.0

–

9 (32.1%)

 Cytopenia (≥ 2 cell lineages)

  Yes

–

25 (89.3%)

  No

–

3 (10.7%)

HLH indexes, N (%)

 Hypertriglyceridemia > 3 mmol/L

0.4–1.7

15 (53.6%)

 Hypofibrinogenemia < 1.5 g/L

2.0–4.0

17 (60.7%)

 Hyperferritin > 500 ng/mL

6–159

4 (14.3%)

 Soluble CD25 > 6400 pg/mL

< 6400

28 (100%)

 Low or absent NK cell activity^a

≥ 15.11%

18 (72.0%)

 Hemophagocytosis in bone marrow

–

13 (46.4%)

 Fever (T > 38.5℃, ≥ 7 days)

< 37.4

28 (100%)

 Splenomegaly

–

26 (92.9%)

Inflammatory and cytokine indexes

 ESR (mm/h)

0–15

2.5 (1.0–104.0)

 CRP (mg/L)

0–10

45.0 (5.0–139.0)

 γ-IFN (pg/mL)

1.6–17.3

1.8 (0–44.7)

 TNF (pg/mL)

0.1–5.2

1.29 (0–70.5)

 IL-10 (pg/mL)

2.6–4.9

11.6 (3.3–77.6)

 IL-6 (pg/mL)

1.7–16.6

59.3 (2.4–1356.1)

Liver function indexes

 TBIL (µmol/L)

3.4–20.5

17.3 (4.4–45.3)

 ALB (g/L)

35–55

29.0 (16.1–37.7)

 ALT (U/L)

5–40

12.8 (3.5–143.7)

 AST (U/L)

5–40

32.6 (10.1–203.2)

 r-GT (U/L)

5–50

28.8 (5.0–293.7)

 LDH (U/L)

110–295

317.5 (106.0–988.0)