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Table 2 Diagnostic criteria fulfillment and HLH therapies among 160 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

  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
Diagnostic criteria
 Fever 159 (99.4) 108 (100) 45 (97.8) 0.2987 18 (100) 1
 Splenomegaly 140 (87.5) 91 (84.3) 43 (93.5) 0.1887 16 (88.9) 1
 Bicytopenia (≥2/3 lineages) 148 (92.5) 97 (89.8) 45 (97.8) 0.1098 17 (94.4) 1
 Hypertriglyceridemia or hypofibrinogenemi 154 (96.3) 105 (97.2) 44 (95.7) 0.6353 18 (100) 1
 Hemophagocytosis
  Yes 103 (64.4) 75 (69.4) 24 (52.2) 0.1126 8 (44.4) 0.0283 
  No 47 (29.4) 28 (25.9) 17 (37.0)   10 (55.6)  
  Missing/not done 10 (6.3) 5 (4.6) 5 (10.9)   0  
 Ferritin ≥500 μg/L 149 (93.1) 98 (90.7) 46 (100) 0.0334 18 (100) 0.6144
 Low/absent NK-cell activity d
  Yes 94 (58.8) 69 (63.9) 22 (47.8)   7 (38.9)  
  Missing/not done 66 (41.3) 39 (36.1) 24 (52.2)   11 (61.1)  
 Soluble CD25 ≥ 2400 U/ml d
  Yes 39 (24.4) 34 (31.5) 4 (8.7)    
  No 3 (1.9) 3 (2.8) 0    
  Missing/not done 118 (73.8) 71 (65.7) 42 (91.3)   18 (100)  
HLH therapies
 Etoposide 105 (65.6) 89 (82.4) 15 (32.6) < 0.0001 1 (5.6) < 0.0001
 Cyclosporine A 115 (71.9) 87 (80.6) 26 (56.5) 0.0029 8 (44.4) 0.006
 Dexamethasone 126 (78.8) 93 (86.1) 30 (65.2) 0.0045 8 (44.4) 0.0007
 Methylprednisolone 14 (8.8) 10 (9.3) 4 (8.7) 1 1 (5.6) 1
 Intrathecal therapy 14 (8.8) 12 (11.1) 2 (4.3) 0.2322 0 0.3705
 HLH therapies started before indicated hospital admission 10 (6.3) 8 (7.4) 2 (4.3) 0.7241 1 (5.6) 1
  1. a. Six patients were lost follow-up at day 30
  2. b. Compared with patients survived > 30 days
  3. c. Compared with patients survived > 7 days
  4. d. Between-group comparisons were not conducted due to missing value > 30%