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Table 3 Underlying diseases and concomitant diagnosis of 160 HLH patients according to survival groups

From: Clinical profiles and risk factors of 7-day and 30-day mortality among 160 pediatric patients with hemophagocytic lymphohistiocytosis

Diagnosis

All (N = 160)

Survival days a

> 30 days (n = 108)

≤30 days (n = 46)

≤7 days (n = 18)

n(%)

n(%)

n(%)

p value b

n(%)

p value c

Underlying/related diseases

 Primary HLH

12 (7.5)

9 (8.3)

3 (6.5)

1

3 (16.7)

0.1375

 Autoimmune disorders

4 (2.5)

3 (2.8)

1 (2.2)

1

0

1

 Malignancy

8 (5.0)

6 (5.6)

2 (4.3)

1

1 (5.6)

1

 EBV infection

88 (55.0)

65 (60.2)

20 (43.5)

0.0763

5 (27.8)

0.0137

 Sepsis

97 (60.6)

55 (50.9)

39 (84.8)

0.0001

17 (94.4)

0.0018

 -Sepsis with confirmed infections

86 (53.8)

53 (49.1)

32 (69.6)

< 0.0001

11 (61.1)

< 0.0001

 -Sepsis with EBV infection

48 (30.0)

31 (28.7)

16 (34.8)

0.4520

4 (22.2)

0.4447

 Other infections d

45 (28.1)

36 (33.3)

7 (15.2)

0.0300

1 (5.6)

0.0238

Concomitant diagnosis

 Myocardial damage

58 (36.3)

23 (21.3)

35 (76.1)

< 0.0001

16 (88.9)

< 0.0001

 Heart failure

28 (17.5)

10 (9.3)

18 (39.1)

< 0.0001

8 (44.4)

0.0043

 Shock

20 (12.5)

5 (4.6)

15 (32.6)

< 0.0001

10 (55.6)

< 0.0001

 CNS disease

39 (24.4)

16 (14.8)

23 (50.0)

< 0.0001

9 (50.0)

0.016

 Hepatic dysfunction

96 (60.0)

53 (49.1)

41 (89.1)

< 0.0001

18 (100)

0.0002

 Respiratory failure

64 (40.0)

26 (24.1)

38 (82.6)

< 0.0001

16 (88.9)

< 0.0001

 ARDS

13 (8.1)

3 (2.8)

10 (21.7)

0.0003

4 (22.2)

0.0423

 Severe pneumonia

61 (38.1)

25 (23.1)

36 (78.3)

< 0.0001

13 (72.2)

0.0016

 Coagulopathy

83 (51.9)

39 (36.1)

41 (89.1)

< 0.0001

18 (100)

< 0.0001

 DIC

42 (26.3)

16 (14.8)

25 (54.3)

< 0.0001

12 (66.7)

0.0002

 Acute kidney injury

17 (10.7)

5 (4.7)

12 (26.1)

0.0003

7 (38.9)

0.0007

 Gastrointestinal disorder

31 (19.4)

8 (7.4)

23 (50.0)

< 0.0001

8 (44.4)

0.0089

 MODS

83 (52.2)

40 (37.4)

42 (91.3)

< 0.0001

18 (100)

< 0.0001

 Immunodeficiency

37 (23.1)

18 (16.7)

19 (41.3)

0.0018

6 (33.3)

0.3714

  1. ARDS = acute respiratory distress syndrome. CNS disease = central nervous system disease. DIC = disseminated intravascular coagulation. EBV = Epstein-Barr Virus. MODS = multiple organ dysfunction syndrome
  2. a. Six patients were lost follow-up at day 30
  3. b. Compared with patients survived > 30 days
  4. c. Compared with patients survived > 7 days
  5. d. Included non-sepsis patients that had any pathogens identified other than EBV