Table 2.
Presentation of the 180 Patients According to Whether They Fulfilled the Hemophagocytic Lymphohistiocytosis-2004 Diagnostic Criteria or Not or Had <4 Criteria Recorded
| Number of Fulfilled HLH-2004 Criteria ≥4 (n = 31) | Number of Fulfilled HLH-2004 Criteria <4 (n = 46) | Number of Recorded HLH-2004 Criteria <4 (n = 103) | |
|---|---|---|---|
| Female | 20 (64.5)/11 (35.5)a | 14 (30.4)/32 (69.6) | 43 (41.7)/60 (58.3) |
| Dead | 12 (38.7)/19 (61.3) | 11 (23.9)/35 (76.1) | 16 (15.5)/87 (84.5) |
| Splenomegaly | 2 (6.7)/28 (93.3); 1 | 2 (4.4)/43 (95.6); 1 | 3 (3.9)/73 (96.1); 27 |
| Hepatomegaly | 8 (26.7)/22 (73.3); 1 | 12 (26.7)/33 (73.3); 1 | 13 (17.1)/63 (82.9); 27 |
| Hemophagocytosis | 13 (86.7)/2 (13.3); 16 | 3 (60.0)/2 (40.0); 41 | 0 (0.0)/0 (0.0); 103 |
| Leak | 19 (61.3)/12 (38.7) | 26 (56.5)/20 (43.5) | 63 (61.2)/40 (38.8) |
| Bleed | 10 (32.3)/21 (67.7) | 14 (30.4)/32 (69.6) | 40 (38.8)/63 (61.2) |
| Severe organ involvement | 26 (83.9)/5 (16.1) | 31 (67.4)/15 (32.6) | 42 (42.4)/57 (57.6); 4 |
| Severe organ involvement (liver) | 24 (77.4)/7 (22.6) | 20 (43.5)/26 (56.5) | 24 (24.5)/74 (75.5); 5 |
| Severe organ involvement (CNS) | 6 (19.4)/25 (80.6) | 11 (23.9)/35 (76.1) | 18 (17.5)/85 (82.5) |
| Severe organ involvement (heart and other) | 11 (35.5)/20 (64.5) | 9 (19.6)/37 (80.4) | 18 (17.5)/85 (82.5) |
| Intubation and ventilation | 19 (61.3)/12 (38.7) | 18 (39.1)/28 (60.9) | 33 (32.0)/70 (68.0) |
| Inotropic support | 15 (48.4)/16 (51.6) | 15 (32.6)/31 (67.4) | 25 (24.3)/78 (75.7) |
| Continuous veno-venous hemodiafiltration | 12 (38.7)/19 (61.3) | 10 (21.7)/36 (78.3) | 10 (9.7)/93 (90.3) |
| Corticosteroid treatment | 15 (48.4)/16 (51.6) | 8 (17.4)/38 (82.6) | 2 (1.9)/101 (98.1) |
| Age at hospital admission, y | 36.4 (20.9–77.7)b | 41.45 (19.3–84.3) | 33.7 (18.2–75.3) |
| Length of hospitalization, d | |||
| Survivors | 6.1 (3.1–25.9) | 5.9 (2.7–60.1) | 6.2 (3.2–37) |
| Nonsurvivors | 3.9 (1.3–15.0) | 2.3 (0.5–43.7) | 3.3 (0.2–22.1) |
| Length of ICU, d | |||
| Survivors | 3.1 (0.9–13.5) | 2.4 (0.3–60) | 2.7 (0.2–34.6) |
| Nonsurvivors | 2.2 (0.7–14.5) | 2.0 (0.3–43.4) | 2.5 (0.1–21.7) |
| Lowest platelets, ×109/L | 14 (1–35) | 9 (0–91) | 18 (1–154) |
| Peak triglycerides, mmol/L | 2.51 (0.87–8.73); 7 | 2.25 (0.7–11.2); 19 | 2.42 (0.97–9.7); 91 |
| Lowest fibrinogen, g/L | 1.95 (1–3.2); 13 | 2.8 (1.3–5.7); 31 | 1.7 (1.6–2.8); 100 |
| Peak ferritin, μg/L | 26 603 (816 to >100 000); 2 | 18 893 (565 to >100 000); 9 | 22 236 (3758–83 500); 93 |
| Peak aspartate aminotransferase, U/L | 2590 (109–30 649) | 760.5 (23–27 520) | 300 (22–35 427); 4 |
| Peak alanine aminotransferase, U/L | 890 (23–8330) | 256 (16–6442) | 169.5 (12–4304); 1 |
| Peak lactate dehydrogenase, U/L | 2913 (327–19 987) | 1366 (267–21 591); 1 | 824 (249–11 117); 6 |
| Peak creatinine, μmol/L | 130 (50–850) | 110 (50–350) | 90 (20–1440) |
| APACHE II score | 14 (3–41) | 11 (5–37) | 13 (1–46); 2 |
| SAPS II score | 31 (6–85) | 21.5 (6–82) | 19 (6–106); 2 |
| SOFA score | 8 (2–18) | 7 (0–19) | 5 (1–20); 2 |
Data from Henter et al [21].
Abbreviations: APACHE, Acute Physiology, Age, Chronic Health Evaluation; CNS, central nervous system; HLH, hemophagocytic lymphohistiocytosis; ICU, intensive care unit; SAPS, Simplified Acute Physiology Score; SOFA, Sequential Organ Failure Assessment.
aNo. (%) yes/no; no. missing (if applicable).
bMedian (range); no. missing (if applicable).