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Table 1 The demographic, clinical and laboratorial characteristics of the patients included in the study

From: Development and validation of the prognostic value of ferritin in adult patients with Hemophagocytic Lymphohistiocytosis

Characteristics

Test Cohort (n = 161)

Validation Cohort (n = 68)

All (n = 229)

P-value∗

IAHS

(n = 57)

MAHS

(n = 39)

MCTD

(n = 8)

Mixed

(n = 27)

Unclear

(n = 30)

IAHS

(n = 17)

MAHS

(n = 14)

Mixed

(n = 23)

Unclear

(n = 14)

Test cohort (n = 161)

Validation cohort (n = 68)

General

Gender (male/female), n

37/20

24/15

3/5

16/11

13/17

10/7

9/5

14/9

5/9

93/68

38/30

0.793

Median age (range), y

52(18–88)

46(18–68)

44(19–59)

51(22–78)

44(18–74)

49(20–86)

59(21–76)

62(19–88)

54(47–78)

49(18–88)

55(19–88)

0.022

Clinical features

Fever,n

53

37

7

25

30

16

13

22

13

152

64

1.000

Mean Tmax,°C

39.4

39.4

39.7

39.4

39.4

39.1

39.2

39.3

39.4

39.4

39.3

0.109

Hepatomegaly

8

9

0

6

9

2

0

2

4

32

8

0.140

Splenomegaly

14

15

0

10

12

2

6

4

5

51

17

0.312

Lymph node enlargement

8

11

1

8

7

1

2

4

2

35

9

0.136

Rash

5

4

4

2

10

3

1

2

1

25

7

0.297

Jaundice

13

5

1

5

7

3

1

2

4

31

10

0.412

Edema

10

5

3

5

6

5

2

6

4

29

17

0.228

Bone marrow Hemophagocytosis

41/45

33/37

5/7

17/26

24/26

14/14

11/12

19/19

10/12

120/141

54/57

0.060

Laboratory data

Ferritin ≥ 500 μg/L

49/54

33/37

8/8

23/24

29/30

16/16

12/14

22/23

11/12

142/153

61/65

1.000

EBV DNA copies (+)

23/45

11/28

0/7

12/26

0/25

6/9

5/10

10/15

1/9

46/131

22/43

0.061

FIB < 1.5 g/L

15/51

11/33

1/6

4/24

3/27

4/14

4/13

3/20

2/8

34/141

13/55

0.944

ANC < 1.0 × 109/L

12/56

9/36

0/8

7/25

2/29

3/17

5/14

7/23

4/13

30/154

19/67

0.144

Hb < 90 g/L

25/56

19/38

1/8

12/25

6/30

4/17

6/14

9/23

6/13

63/157

25/67

0.693

Platelets

<100 × 109/L

47/56

31/39

4/8

21/25

21/30

15/17

12/14

15/23

10/13

124/158

52/67

0.885

ALT > 40 U/L

43/56

29/38

7/8

13/25

21/29

10/16

7/14

17/23

9/13

113/156

43/66

0.278

AST > 40 U/L

44/56

30/38

7/8

18/25

22/28

12/16

11/14

17/23

9/13

121/155

49/66

0.537

LDH > 271 U/L

47/56

35/39

7/8

21/25

26/29

12/16

12/13

21/23

12/12

136/157

57/64

0.621

DB > 6.8 μmol/L

31/56

18/37

2/8

18/25

14/29

10/16

4/13

15/23

9/12

83/155

39/66

0.448

TG > 3 mmol/L

6/56

13/35

1/8

8/25

4/29

2/16

2/13

5/22

7/12

32/153

16/63

0.471

Albumin < 40 g/L

54/56

35/35

8/8

24/25

29/30

16/16

12/13

23/23

11/12

150/154

62/64

1.000

Ca2+ < 2.20 mmol/L

48/56

26/34

8/8

22/25

24/29

16/17

14/14

21/23

13/13

128/152

64/67

0.019

  1. ALT Alanine aminotransferase, ANC Absolute neutrophil count, AST Aspartate aminotransferase, Ca2+ Calcium, DB Direct bilirubin, EBV Epstein Barr virus, FIB Fibrinogen, HB Hemoglobin, IAHS Infection-associated HLH, MAHS Malignancy-associated HLH, LDH Lactate dehydrogenase, MCTD Mixed connective tissue disease, Mixed Mixed cause HLH, TG Triglyceride, Unclear Unknown underlying diseases; *Comparison with test cohort and validation cohort
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Orphanet Journal of Rare Diseases

ISSN: 1750-1172