Table 1 Clinical presentations and Laboratory data at diagnosis in those patients

Gender

Male

Male

Female

Age of onset (year)

3

1

4

 Family history

–

–

–

 Fever

+

+

–

 Malar rash

+

–

+

 Lymphadenopathy

+

+

–

 Hepatomegaly

+

+

+

 Splenomegaly

–

+

+

 Arthritis

+

+

+

 Pericardial effusion

+

–

–

 Proteinuria

–

–

+

 WBC(×10⁹/L)

5.16–11.73

4.01–8.38

3.45–10.44

3.5–9.5

 Neutrophil (%)

37.5–64.3

53.6–76.3

40.6–82.1

50.0–75.0

 Lymphocyte (%)

24–45.7

17.8–38.6

10.7–29.9

20.0–40.0

 CD3+ cells (%, cells/ml)

79.9, 2335

86.6, 1438

NA

60.05–74.08, 1424–2664

 CD3 + CD4+ cells

35, 1023

40.9, 679

NA

26.17–40.76, 686–1358

 CD3 + CD8+ cells

40.8, 1193

36.4, 604

NA

19.68–34.06, 518–1125

 CD20+ B cells

13.8, 403

6.1, 101

NA

10.21–20.12, 280–623

 Double negative T cells (%) (TCRαβ+CD3 + CD4-CD8-)

4.9

7.2

NA

0.18–2.81

 Monocyte (%)

8.8–18.3

2.4–10.9

6.9–28.6

3.0–8.0

 Monocyte

0.69–1.79

0.2–0.83

0.45–2.4

0.12–0.80

 Hemoglobin (g/dL)

5.7–10.4

12.1–15.8

10.5–15.7

12.0–16.0

 RET%

4.07

1

1.46

0.8–2.0

 Platelet (×10⁹/L)

24–366

28–171

14–189

100–350

 CRP (mg/dL)

< 0.1

0.3

< 0.1

0–0.8

 ESR (mm/h)

51

19

31

0–15 for boys

0–20 for girls

 Double negative T cells (%) (TCRαβ+CD3 + CD4-CD8-)

4.9

7.2

NA

 IgG (mg/dL)

2331

1629

2158

500–1200

 IgM (mg/dL)

625

201

56

40–230

 IgA (mg/dL)

312

133

743

70–400

 C3 (g/L)

< 0.406

0.251

↓

0.73–1.46

 C4 (g/L)

< 0.046

0.010

↓

0.1–0.4

 ANA

H1:1280

H1:640

S1:1280

< 1:40

 Anti-dsDNA-ELISA (U/mL)

664

770

140

< 100

 Anti-dsDNA-IF

+ 1:320

+ 1:40

–

< 1:5

 Anti-Sm

–

–

+(1:4)

–

 ACL (U/mL)

17

–

–

< 12

 β2GP1-IgA (U/mL)

54

–

–

< 20

 Coombs

+

+

+

–

 EB IgG/VCA

+  5.62

+  7.93

NA

< 0.80

 EB IgM/VCA

+  1.31

–

NA

< 0.80

 EBV-DNA

+

–

NA

< 500

 CMV-IgM

+  3.18

+  1.86

+  1.31

< 0.9

 CMV-IgG

NA

+  3.64

+  4.79

< 0.9

 CMV-PP65

+

+

–

–