Table 1 Patient’s laboratory and clinical findings during his clinical course

Parameters for HLH diagnosis

 High fever

+

+

-

-

 Splenomegaly (under ribs, cm)

4

4.5

-

-

 Neutrophil count (×10⁹/L)

3.21

1.75

6.08

0.72–4.6

 Platelet count (×10⁹/L)

139

89

116

100–400 × 10⁹

 Hemoglobin (g/L)

82

76

284

110–190

 Fibrinogen (g/L)

2.2

1.24

2.44

2–4

 Triglyceride (mmol/L)

1.52

3.51

1.31

0–2

 Serum ferritin (µg/L)

175.7

269.6

163

28–397

 Soluble CD25(pg/mL)

ND

131,840

4117

0–6400

 Hemophagocytosis in BM

-

ND

ND

-

 NK cell activity (%)

ND

12.81

14.32

>15.44

Other indicators

 Neurological manifestations

+

+, deterioration

-

-

 CSF pressure (mmH₂O)

230

>330

ND

40–100

 CSF cell count (×10⁶/L)

30

45

ND

0–15 × 10⁶

 CSF Protein (mg/L)

743

1216

ND

20–350

 Head CT scan

Low density of brain parenchyma

Extensive lesions, cerebral edema, tonsil hernia

Greatly improved, cerebral edema (-) tonsil hernia (-)

-

 IFN-γ(pg/ml)

ND

213.6

9.83

1.60–17.30

 NK cell CD107a (%)

ND

1.01

2.33

>10

Infection, malignancy or autoimmune disease

-

-

ND

-