Table 2 Diagnostic criteria fulfillment and HLH therapies among 160 patients according to survival groups
|  | 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 |