Table 2.
| Variable | All-cause mortality (n=80) |
|
|---|---|---|
| Univariate HR (95% CIs) | P | |
| Epidemiological variables | ||
| Age at diagnosis (y) | 1.03 (1.02-1.04) | <.001 |
| Sex: male | 1.61 (1.01-2.56) | .04 |
| Underlying disease grouped | ||
| None | Reference | |
| Neoplasia | 1.84 (1.01-3.37) | .05 |
| Autoimmune/rheumatic disease | 0.34 (0.16-0.74) | .006 |
| Others | 0.98 (0.45-2.17) | .97 |
| Multiple (more than 1) | 1.24 (0.66-2.35) | .51 |
| Active ID therapies | 0.76 (0.45-1.28) | .30 |
| Clinical variables | ||
| Fever (>37.5°C) | 0.93 (0.43-2.03) | .86 |
| Splenomegaly | 0.88 (0.56-1.39) | .59 |
| Hepatomegaly | 1.24 (0.80-1.92) | .34 |
| Peripheral adenopathies | 0.63 (0.40-0.98) | .04 |
| Pulmonary involvement | 1.40 (0.91-2.18) | .13 |
| CNS involvement | 1.11 (0.64-1.91) | .71 |
| Skin lesions | 0.82 (0.50-1.37) | .45 |
| Gastrointestinal involvement | 1.03 (0.65-1.65) | .89 |
| Renal involvement | 1.33 (0.84-2.10) | .23 |
| Hemoglobin <9 g/dL | 1.71 (0.96-3.05) | .07 |
| Platelets <100,000 cells per mm3 | 3.39 (1.37-8.40) | .008 |
| WBC <4000 cells per mm3 | 1.81 (1.01-3.23) | .05 |
| Neutrophils <1000 cells per mm3 | 1.48 (0.90-2.42) | .12 |
| Triglycerides >150 mg/dL | 1.24 (0.54-2.86) | .61 |
| Natremia <130 mmol/L | 1.61 (1.02-2.54) | .04 |
| AST transaminase >100 IU/L | 0.89 (0.57-1.40) | .62 |
| ALT transaminase >100 IU/L | 0.81 (0.52-1.25) | .34 |
| Lactate dehydrogenase >450 IU/L | 0.84 (0.54-1.31) | .45 |
| Ferritin >500 ng/mL | 3.05 (0.42-21.97) | .27 |
| Fibrinogen <1.5 g/L | 1.29 (0.76-2.22) | .35 |
| Disseminated intravascular coagulation | 1.87 (1.05-3.34) | .03 |
| Histopathological hemophagocytosis | 1.71 (0.63-4.68) | .30 |
| HScore (≥169) | 1.29 (0.59-2.84) | .53 |
| Infectious trigger | ||
| Presence of infectious trigger | 1.53 (0.96-2.45) | .07 |
| Microbiological classification | ||
| No infection | Reference | |
| Bacteria | 1.99 (1.09-3.63) | .03 |
| Virus | 1.36 (0.79-2.34) | .28 |
| Fungi/parasites | 0.78 (0.27-2.24) | .65 |
| Mixed | 3.42 (1.38-8.46) | .008 |
| Number of infectious triggers | ||
| 0 | Reference | |
| 1 | 1.38 (0.85-2.24) | .20 |
| ≥2 | 2.95 (1.43-6.08) | .003 |
| Therapeutic interventions | ||
| No immunosuppressive drugs | Reference | |
| Glucocorticoid monotherapy | 1.21 (0.60-2.44) | .60 |
| ID/IVIG/biological therapies | 0.44 (0.20-0.96) | .04 |
| Chemotherapy | 0.69 (0.34-1.39) | .30 |
a
ALT = alanine aminotransferase; AST = aspartate aminotransferase; CNS = central nervous system; HR = hazard ratio; ID = immunosuppressive drug; IVIG = intravenous immunoglobulin; WBC = white blood cell.
b
Values are represented as HRs and 95% CIs.