Table 1.
Demographic and clinical information of patients with EBV-HLH in the primary cohort.
| Characteristics | Deathn = 54 | Survivaln = 130 | P-value |
|---|---|---|---|
| Male, no. (%) | 29 (53.7%) | 86 (66.2%) | 0.113 |
| Age >18 years, no. (%) | 44 (81.4%) | 79 (60.8%) | 0.007┼ |
| Therapy strategy, no. (%) | 0.206 | ||
| Etoposide-based | 40 (74.1%) | 107 (82.3%) | |
| No etoposide-based | 14 (25.9%) | 23 (17.7%) | |
| Infection history, no. (%) | |||
| Tuberculous | 12 (22.2) | 14 (10.8%) | 0.043┼ |
| Other | 50 (92.5%) | 111 (85.4%) | 0.179 |
| Brain involvement, no. (%) | 18 (33.3%) | 12 (1.5%) | <0.001┼ |
| Liver/spleen involvement, no. (%) | |||
| Splenomegaly | 18 (33.3%) | 60 (46.2%) | 0.110 |
| Hepatomegaly | 1 (1.9%) | 4 (3.1%) | 0.643 |
| Hepatosplenomegaly | 28 (51.9%) | 47 (36.2%) | 0.049┼ |
| Laboratory findings | |||
| WBC count, m (IQR), ×109 | 1.5 (0.9–2.5) | 2.1 (1.4–3.6) | 0.015┼ |
| Hemoglobin (g/l), m (IQR) | 87 (77–100) | 101.5 (84–119) | 0.001┼ |
| Platelet count, m (IQR), ×109 | 45 (24–68) | 60.5 (37–118) | 0.007┼ |
| ALT (U/L), m (IQR) | 91.5 (63.0–183.0) | 81.5 (42.0–188.0) | 0.455 |
| AST (U/L), m (IQR) | 145.2 (52.5–257.0) | 84.9 (43.0–193.0) | 0.070 |
| GGT (U/L), m (IQR) | 202 (111–378) | 124 (48–282) | 0.007┼ |
| ALP (U/L), m (IQR) | 290 (129–566) | 207 (100–400) | 0.049┼ |
| Tbi (μmol/L), m (IQR) | 32.0 (14.2–62.4) | 19.4 (12.3–34.0) | 0.031┼ |
| Dbi (μmol/L), m (IQR) | 17.2 (5.0–58.1) | 7.4 (3.5–20.0) | 0.004┼ |
| Ibi (μmol/L), m (IQR) | 12.9 (8.3–27.3) | 12.0 (7.1–15.5) | 0.055 |
| ALB (g/L), m (IQR) | 27.2 (24.0–32.9) | 31.7 (27.7–37.4) | <0.001┼ |
| Cr (μmol/L), m (IQR) | 55.2 (44.0–78.3) | 53.7 (44.2–69.4) | 0.455 |
| Bun (mmol/L), m (IQR) | 5.7 (4.2–7.5) | 5.0 (3.7–7.1) | 0.038┼ |
| K+ (mmol/L), m (IQR) | 3.92 (3.61–4.10) | 4.04 (3.72–4.39) | 0.052 |
| Ca2+ (mmol/L), m (IQR) | 1.97 (1.78–2.11) | 2.10 (1.93–2.23) | 0.002┼ |
| Na+ (mmol/L), m (IQR) | 134.0 (132.1–137.8) | 136.9 (133.2–139.6) | 0.013┼ |
| Fibrinogen (g/L), m (IQR) | 1.26 (0.77–1.79) | 1.61 (1.02–2.47) | 0.018┼ |
| PCT > 2 µg/L, no. (%) | 15 | 16 | 0.027┼ |
| ESR (mm/H), m (IQR) | 27 (10–30) | 18 (10–30) | 0.845 |
| Specific findings | |||
| Hemophagocytosis, no. (%) | 41 (75.9%) | 105 (80.8%) | 0.461 |
| Serum ferritin (ng/ml), m (IQR), ×103 | 2.82 (1.65–15.00) | 2.20 (0.95–13.07) | 0.078 |
| sCD25 (pg/ml)†, m (IQR), ×104 | 2.52 (1.94–4.40) | 2.18 (0.31–4.01) | 0.019┼ |
| The activity of NK cells (%)‡, m (IQR) | 14.4 (13.0–14.5) | 14.4 (13.5–16.4) | 0.085 |
| EBV-DNA (copies/ml), m (IQR), ×105 | |||
| Plasma | 3.8 (1.5–17.0) | 3.8 (0.3–26.0) | 0.530 |
| PBMC | 8.7 (4.6–100.0) | 6.2 (0.8–21.0) | 0.007┼ |
| NK-CD107a (%), m (IQR) | 12.2 (10.4–12.2) | 12.2 (10.7–20.3) | 0.198 |
| CTL-CD107a (%), m (IQR) | 2.7 (2.7–3.4) | 2.7 (2.2–3.3) | 0.247 |
┼The parameter was significant.
†The normal range of sCD25 is <6,400.
‡The normal range of the activity of NK cells is ≥15.11%.
WBC, white blood cell; ALT, glutamic-pyruvic transaminase; AST, glutamic oxalacetic transaminase; GGT, gamma-glutamyl transpeptidase; ALP, alkaline phosphatase; Tbi, total bilirubin; Dbi, direct bilirubin; Ibi, indirect bilirubin; ALB, albumin; Cr, creatinine; Bun, blood urea nitrogen; PCT, procalcitonin; ESR, erythrocyte sedimentation rate; serum CD25; NK cells, nature killer cells; EBV, Epstein–Barr virus; DNA, deoxyribonucleic acid; PBMC, peripheral blood mononuclear cell; CTL, cytotoxic T lymphocyte.