Table 2.
Basic patient characteristics, biomarkers, and outcome parameters
| Parameters | HLH patients (n = 40) | Non-HLH patients (n = 2583) | P value |
|---|---|---|---|
| Age [years] | 47 (33–62) | 62 (49–73) | < 0.001‡ |
| Male sex [n] (%) | 26 (65.0%) | 1588 (61.5%) | 0.650† |
| Body mass index [kg/m2] | 23.0 (21.0–26.5) | 25.0 (22.0–29.0) | 0.094‡ |
| Sepsis without shock [n] (%) | 12 (30.0%) | 1003 (38.8%) | 0.255† |
| Septic shock [n] (%) | 23 (57.5%) | 626 (24.2%) | < 0.001† |
| Hemodialysis [n] (%) | 29 (72.5%) | 1357 (52.5%) | 0.012† |
| ECLA/ECMO [n] (%) | 6 (15.0%) | 188 (7.3%) | 0.064† |
| ICU admission SOFA score | 9 (6–13) | 6 (3–9) | < 0.001‡ |
| Maximum SOFA score | 17 (12–19) | 11 (7–15) | < 0.001‡ |
| HLH-2004 criteria | |||
| Measured | 7 (6–7) | 4 (4–5) | < 0.001‡ |
| Fulfilled | 5 (4–6) | 2 (1–2) | < 0.001‡ |
| HScore | 258 (225–280) | 62 (33–101) | < 0.001‡ |
| Bi-/pancytopenia [n] (%)*, n = 40|2582 | 37 (92.5%) | 471 (18.2%) | < 0.001† |
| Hemoglobin [g/dL] | 6.9 (6.4–7.6) | 8.8 (7.9–9.9) | < 0.001‡ |
| Platelet count [/nL] | 18 (5–34) | 170 (88–270) | < 0.001‡ |
| Leukocyte count [/nL] | 0.9 (0.2–2.7) | 9.0 (6.1–13.3) | < 0.001‡ |
| Hypofibrinogenemia or hypertriglyceridemia [n] (%), n = 40|1517 | 30 (75.0%) | 288 (19.0%) | < 0.001† |
| Fibrinogen [mg/dL], n = 39|1144 | 2.0 (1.0–3.0) | 3.8 (2.5–5.3) | < 0.001‡ |
| Triglycerides [mg/dL], n = 39|855 | 376 (245–563) | 158 (104–247) | < 0.001‡ |
| Max. core body temperature [°C], n = 40|2452 | 39.1 (38.5–39.8) | 38.2 (37.5–38.9) | < 0.001† |
| Splenomegaly [n] (%), n = 40|2008 | 26 (65.0%) | 401 (20.0%) | < 0.001† |
| Hepatomegaly [n] (%), n = 40|2037 | 23 (57.5%) | 328 (16.1%) | < 0.001† |
| Hemophagocytosis [n] (%), n = 31|221 | 16 (51.6%) | 15 (6.8%) | < 0.001† |
| AST [U/L], n = 40|2327 | 171 (119–498) | 47 (26–108) | < 0.001‡ |
| Pre-existing immunosuppression [n] (%) | 30 (75.0%) | 728 (28.2%) | < 0.001† |
| ICU duration [d] | 20.0 (11.3–37.3) | 19.0 (6.0–47.1) | 0.522‡ |
| In-patient duration [d] | 27.7 (18.6–77.4) | 38.3 (18.1–76.1) | 0.682‡ |
| Deceased [n] | 24 (60.0%) | 741 (28.7%) | < 0.001† |
Diagnostic parameters with n representing the number of patients with available data in each group, if not available in all patients; continuous quantities in median with quartiles. CD107a testing as a functional marker for identification of NK cell activity was performed in 4 patients only; however, three showed values within the normal range and one could not be analyzed due to low NK cell count. Values of ferritin and sIL-2R of the cohort were already described in Lachmann et al. [8]
ECLA extracorporeal lung assist, ECMO extracorporeal membrane oxygenation, ICU intensive care unit, SOFA sequential organ failure assessment
‡P values calculated using the Mann-Whitney U test
†P values calculated using the χ2 test
*Leukopenia was assumed by white blood cell count < 1.67/nL