Clinical features of patients with EBV-HLH suitable for HSCT
| . | Total (n = 199) . | Transplanted group (n = 88) . | Nontransplanted group (n = 111) . | P value . |
|---|---|---|---|---|
| Age, y | 29 (18-59) | 25 (18-58) | 33 (18-59) | <.001∗∗ |
| Subtype, acute EBV-HLH/CAEBV-HLH | 157/42 | 67/21 | 90/21 | .396 |
| Sex, M/F | 129/70 | 58/30 | 71/40 | .881 |
| Coagulation abnormalities, Y/N | 92/86 | 33/34 | 59/52 | .614 |
| WBC, ×109/L | 2.39 (0.07-16.95) | 2.57 (0.32-15.60) | 2.11 (0.07-16.95) | .394 |
| ANC, ×109/L | 1.42 (0.00-13.70) | 1.5 (0.03-10.34) | 1.34 (0-13.7) | .728 |
| Hb, g/L | 97 ± 22 | 99 ± 25 | 96 ± 20 | .279 |
| PLT, ×109/L | 83 (1-454) | 89 (11-454) | 75 (1-298) | .082 |
| ALT, U/L | 74 (6-861) | 82 (11-861) | 60 (6-715) | .018∗ |
| AST, U/L | 63.6 (3.2-1376.9) | 87.6 (3.2-894) | 56.8 (6.5-1376.9) | .304 |
| ALB, g/L | 31.3 ± 6.3 | 31.7 ± 6.2 | 31.0 ± 6.4 | .404 |
| Cr, μmol/L | 50.4 (23.9-226.8) | 47.1 (23.9-226.8) | 53.4 (24.1-185.2) | .013∗ |
| Bun, mmol/L | 4.9 (1.26-46.4) | 4.67 (1.26-29.16) | 5.24 (1.61-46.4) | .033∗ |
| TG, mmol/L | 2.2 (0.59-9.18) | 2.07 (0.66-9.18) | 2.23 (0.59-8.06) | .333 |
| FIB, g/L | 1.67 (0.39-5.27) | 1.61 (0.39-4.7) | 1.79 (0.39-5.27) | .265 |
| β2M, mg/L | 4.09 (1.25-22.7) | 4.15 (1.36-22.4) | 4.08 (1.25-22.7) | .867 |
| FER, μg/L | 1 862 (17.9-171 633) | 1 483.7 (61.5-75 000) | 2 092.9 (17.9-171 633) | .206 |
| sCD25, pg/mL | 19 963.5 (222-269 830) | 21 495 (222-155 915) | 19 072 (469-269 830) | .690 |
| NK-cell activity, % | 15.03 (6.38-49.75) | 15.06 (6.97-41.66) | 14.72 (6.38-49.75) | 1.000 |
| Plasma EBV, copies/mL | 12 500 (0-7 600 000) | 8 800 (0-7 600 000) | 21 000 (0-7 500 000) | .051 |
| PBMC EBV, copies/mL | 43 000 (0-32 300 000) | 49 200 (0-32 300 000) | 33 000 (0-20 000 000) | .682 |
| IL-2, pg/mL | 3.4 (0.95-25) | 3.5 (0.95-22.82) | 3.3 (1.04-25) | .471 |
| IL-6, pg/mL | 5.5 (0-595.9) | 5.5 (0-201.8) | 5.7 (0-595.9) | .472 |
| IL-10, pg/mL | 2.8 (0-7051) | 2.76 (0.4-1354) | 2.8 (0-7051) | .805 |
| IL-17A, pg/mL | 1.1 (0-199.2) | 1.1 (0-199.2) | 1.2 (0-9.1) | .771 |
| IL-1RA, pg/mL | 189.5 (0-49 095) | 195.5 (0-26 669) | 180.3 (0-49 095) | .576 |
| IFN-γ, pg/mL | 58.8 (0-2 712) | 65.4 (0-953.05) | 49.8 (0-2712) | .370 |
| TNF-α, pg/mL | 5.8 (0.3-87.56) | 5.8 (2.16-78.15) | 5.7 (0.3-87.56) | .434 |
| IL-18, pg/mL | 122.1 (2.6-1 341) | 122.3 (5.11-1 136) | 120.5 (2.6-1 341) | .840 |
| EBV-positive lymphocytes | .471 | |||
| All lineage (NK-dominant) | 20 (13.6%) | 12 (13.6%) | 8 (13.6%) | |
| All lineage | 35 (23.8%) | 24 (27.3%) | 11 (18.6%) | |
| NK | 32 (21.8%) | 16 (18.2%) | 16 (27.1%) | |
| T | 35 (23.8 %) | 23 (26.1%) | 12 (20.3%) | |
| NK/T | 25 (17.0%) | 13 (14.8%) | 12 (20.3%) | |
| EBER (+/–) | 115/61 | 58/26 | 57/35 | .323 |
| . | Total (n = 199) . | Transplanted group (n = 88) . | Nontransplanted group (n = 111) . | P value . |
|---|---|---|---|---|
| Age, y | 29 (18-59) | 25 (18-58) | 33 (18-59) | <.001∗∗ |
| Subtype, acute EBV-HLH/CAEBV-HLH | 157/42 | 67/21 | 90/21 | .396 |
| Sex, M/F | 129/70 | 58/30 | 71/40 | .881 |
| Coagulation abnormalities, Y/N | 92/86 | 33/34 | 59/52 | .614 |
| WBC, ×109/L | 2.39 (0.07-16.95) | 2.57 (0.32-15.60) | 2.11 (0.07-16.95) | .394 |
| ANC, ×109/L | 1.42 (0.00-13.70) | 1.5 (0.03-10.34) | 1.34 (0-13.7) | .728 |
| Hb, g/L | 97 ± 22 | 99 ± 25 | 96 ± 20 | .279 |
| PLT, ×109/L | 83 (1-454) | 89 (11-454) | 75 (1-298) | .082 |
| ALT, U/L | 74 (6-861) | 82 (11-861) | 60 (6-715) | .018∗ |
| AST, U/L | 63.6 (3.2-1376.9) | 87.6 (3.2-894) | 56.8 (6.5-1376.9) | .304 |
| ALB, g/L | 31.3 ± 6.3 | 31.7 ± 6.2 | 31.0 ± 6.4 | .404 |
| Cr, μmol/L | 50.4 (23.9-226.8) | 47.1 (23.9-226.8) | 53.4 (24.1-185.2) | .013∗ |
| Bun, mmol/L | 4.9 (1.26-46.4) | 4.67 (1.26-29.16) | 5.24 (1.61-46.4) | .033∗ |
| TG, mmol/L | 2.2 (0.59-9.18) | 2.07 (0.66-9.18) | 2.23 (0.59-8.06) | .333 |
| FIB, g/L | 1.67 (0.39-5.27) | 1.61 (0.39-4.7) | 1.79 (0.39-5.27) | .265 |
| β2M, mg/L | 4.09 (1.25-22.7) | 4.15 (1.36-22.4) | 4.08 (1.25-22.7) | .867 |
| FER, μg/L | 1 862 (17.9-171 633) | 1 483.7 (61.5-75 000) | 2 092.9 (17.9-171 633) | .206 |
| sCD25, pg/mL | 19 963.5 (222-269 830) | 21 495 (222-155 915) | 19 072 (469-269 830) | .690 |
| NK-cell activity, % | 15.03 (6.38-49.75) | 15.06 (6.97-41.66) | 14.72 (6.38-49.75) | 1.000 |
| Plasma EBV, copies/mL | 12 500 (0-7 600 000) | 8 800 (0-7 600 000) | 21 000 (0-7 500 000) | .051 |
| PBMC EBV, copies/mL | 43 000 (0-32 300 000) | 49 200 (0-32 300 000) | 33 000 (0-20 000 000) | .682 |
| IL-2, pg/mL | 3.4 (0.95-25) | 3.5 (0.95-22.82) | 3.3 (1.04-25) | .471 |
| IL-6, pg/mL | 5.5 (0-595.9) | 5.5 (0-201.8) | 5.7 (0-595.9) | .472 |
| IL-10, pg/mL | 2.8 (0-7051) | 2.76 (0.4-1354) | 2.8 (0-7051) | .805 |
| IL-17A, pg/mL | 1.1 (0-199.2) | 1.1 (0-199.2) | 1.2 (0-9.1) | .771 |
| IL-1RA, pg/mL | 189.5 (0-49 095) | 195.5 (0-26 669) | 180.3 (0-49 095) | .576 |
| IFN-γ, pg/mL | 58.8 (0-2 712) | 65.4 (0-953.05) | 49.8 (0-2712) | .370 |
| TNF-α, pg/mL | 5.8 (0.3-87.56) | 5.8 (2.16-78.15) | 5.7 (0.3-87.56) | .434 |
| IL-18, pg/mL | 122.1 (2.6-1 341) | 122.3 (5.11-1 136) | 120.5 (2.6-1 341) | .840 |
| EBV-positive lymphocytes | .471 | |||
| All lineage (NK-dominant) | 20 (13.6%) | 12 (13.6%) | 8 (13.6%) | |
| All lineage | 35 (23.8%) | 24 (27.3%) | 11 (18.6%) | |
| NK | 32 (21.8%) | 16 (18.2%) | 16 (27.1%) | |
| T | 35 (23.8 %) | 23 (26.1%) | 12 (20.3%) | |
| NK/T | 25 (17.0%) | 13 (14.8%) | 12 (20.3%) | |
| EBER (+/–) | 115/61 | 58/26 | 57/35 | .323 |
Coagulation abnormalities are indicated by prolonged prothrombin time (PT) and/or activated partial thromboplastin time (APTT). Decreased CD107a is defined as a ≤10% difference before and after NK-cell stimulation and/or <2.8 difference before and after cytotoxic T lymphocyte cell stimulation. ∗P < .05; ∗∗P < .001.
ANC, absolute neutrophil count; AST, aspartate transaminase; EBER, EBV-encoded RNA; IFN-γ, interferon gamma; IL-1RA, interleukin-1 receptor antagonist; M/F, male/female; PLT, platelet; TNF- α, tumor necrosis factor α; Y/N, yes/no.