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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