Table 3.
Symptoms and laboratory tests within 48 h of hospital admission according to HLH diagnostic time lag categories.
| Hospital admission to diagnosis | ||||
|---|---|---|---|---|
| All n (%) | ≤3 days n (%) | > 3 days n (%) | P | |
| Total | 122 | 82 | 40 | |
| HLH-2004 criteria† | ||||
| Fever | 117 (95.9) | 80 (97.6) | 37 (92.5) | 0.3292 |
| Splenomegaly | 89 (73.0) | 66 (80.5) | 23 (57.5) | 0.0073 |
| Cytopenias | 82 (67.2) | 67 (81.7) | 15 (37.5) | <0.0001 |
| Hemoglobin <90 g/L | 73 (59.8) | 59 (72.0) | 14 (35.0) | 0.0001 |
| Platelets <100 × 109 /L | 94 (77.0) | 72 (87.8) | 22 (55.0) | 0.0001 |
| Neutrophils <1.0 × 1 09 /L | 65 (53.3) | 51 (62.2) | 14 (35.0) | 0.0047 |
| Hypertriglyceridemia and/or hypofibrinogenemia | 76 (62.3) | 63 (76.8) | 13 (32.5) | <0.0001 |
| Hypertriglyceridemia, ≥ 3.0 mmol/L | ||||
| No | 50 (41.0) | 33 (40.2) | 17 (42.5) | 0.0085 |
| Yes | 41 (33.6) | 34 (41.5) | 7 (17.5) | |
| Not done | 31 (25.4) | 15 (18.3) | 16 (40.0) | |
| Hypofibrinogenemia, ≤ 1.5 g/L | ||||
| No | 45 (36.9) | 29 (35.4) | 16 (40.0) | 0.0005 |
| Yes | 64 (52.5) | 52 (63.4) | 12 (30.0) | |
| Not done | 13 (10.7) | 1 (1.2) | 12 (30.0) | |
| Hemophagocytosis | ||||
| No | 12 (9.8) | 7 (8.5) | 5 (12.5) | <0.0001 |
| Yes | 34 (27.9) | 33 (40.2) | 1 (2.5) | |
| Not done | 76 (62.3) | 42 (51.2) | 34 (85.0) | |
| Low or absent NK-cell activity‡ | ||||
| Yes | 61 (50.0) | 53 (64.6) | 8 (20.0) | |
| Not done | 61 (50.0) | 29 (35.4) | 32 (80.0) | |
| Ferritin ≥ 500 μg/L | ||||
| No | 7 (5.7) | 3 (3.7) | 4 (10.0) | 0.0028 |
| Yes | 37 (30.3) | 32 (39.0) | 5 (12.5) | |
| Not done | 78 (63.9) | 47 (57.3) | 31 (77.5) | |
| No. of fulfilled items from 8 items | ||||
| 0 item | 2 (1.6) | 0 | 2 (5.0) | <0.0001 |
| 1 item | 5 (4.1) | 2 (2.4) | 3 (7.5) | |
| 2 items | 20 (16.4) | 3 (3.7) | 17 (42.5) | |
| 3 items | 28 (23.0) | 14 (17.1) | 14 (35.0) | |
| 4 items | 34 (27.9) | 30 (36.6) | 4 (10.0) | |
| 5 items | 26 (21.3) | 26 (31.7) | 0 | |
| 6 items | 7 (5.7) | 7 (8.5) | 0 | |
| Median (Q1,Q3) | 4 (3, 5) | 4 (4, 5) | 2 (2, 3) | <0.0001 |
| Min, max | 0, 6 | 1, 6 | 0, 4 | |
| Fulfill 3 regularly checked criteria: fever, splenomegaly, and cytopenias | 61 (50.0) | 54 (65.9) | 7 (17.5) | <0.0001 |
| Early diagnostic tests for HLH§ | ||||
| Within 48 h | 19 (15.6) | 17 (20.7) | 2 (5.0) | 0.0245 |
| Within 72 h | 77 (63.1) | 63 (76.8) | 14 (35.0) | <0.0001 |
| Other tests within 48 h¶ | ||||
| TBil, >19 μmol/L | 55 (45.1) | 45 (54.9) | 10 (25.0) | 0.0018 |
| ALT, >40 U/L | 99 (81.1) | 75 (91.5) | 24 (60.0) | <0.0001 |
| APTT, >48s | 74 (60.7) | 62 (75.6) | 12 (30.0) | <0.0001 |
| PT, >14s | 85 (69.7) | 66 (80.5) | 19 (47.5) | 0.0002 |
| INR, >1.5 | 34 (27.9) | 26 (31.7) | 8 (20.0) | 0.1758 |
ALT, Alanine aminotransferase; APTT, activated partial thromboplastin time; HLH, haemophagocytic lymphohistiocytosis; INR, international normalized ratio; NK-cell, natural killer cell; PT, prothrombin time; TBil, total bilirubin.
Values in bold are statistically significant (p <0.05).
†
Results for soluble CD25 tests and genetic tests were not available within 72 h of admission.
‡
p-value not estimated because no negative results was detected.
§
Accomplishing all diagnostic tests from the HLH-2004 criteria except for NK-cell activity, soluble CD25, and genetic tests.
¶
Displayed according to local lab reference ranges.