Demographic and diagnostic information of patients with HLH and partial HLH
| Variables . | Total (N = 1215) . | HLH (n = 627) . | Partial HLH (n = 588) . | P value† . |
|---|---|---|---|---|
| Age, median (IQR), y | 51 (34-63) | 51 (34-63) | 52 (33-63) | .872 |
| Female, n (%) | 645 (53.1) | 309 (49.3) | 336 (57.1) | .007∗∗ |
| HLH triggers, n (%) | ||||
| Malignancy | 285 (23.5) | 207 (33) | 78 (13.3) | <.001∗∗∗ |
| HM | 228 (18.8) | 169 (27) | 59 (10) | <.001∗∗∗ |
| T-/NK-cell lymphoma | 117 (9.6) | 84 (13.4) | 33 (5.6) | <.001∗∗∗ |
| B-cell lymphoma | 78 (6.4) | 62 (9.9) | 16 (2.7) | <.001∗∗∗ |
| Hodgkin lymphoma | 6 (0.5) | 4 (0.6) | 2 (0.3) | .688 |
| Leukemia | 14 (1.2) | 12 (1.9) | 2 (0.3) | .021∗ |
| MDS | 11 (0.9) | 6 (1) | 5 (0.9) | 1 |
| Multiple myeloma | 1 (0.1) | 1 (0.2) | 0 (0) | 1 |
| POEMS syndrome | 1 (0.1) | 0 (0) | 1 (0.2) | .484 |
| Other malignancy | 57 (4.7) | 38 (6.1) | 19 (3.2) | .028∗ |
| Infection‡ | 580 (47.7) | 271 (43.2) | 309 (52.6) | .001∗∗ |
| EBV infection | 505 (41.6) | 310 (49.4) | 195 (33.2) | <.001∗∗∗ |
| EBV with malignancy | 149 (12.3) | 121 (19.3) | 28 (4.8) | <.001∗∗∗ |
| EBV with RHD | 75 (6.2) | 26 (4.1) | 49 (8.3) | .004∗∗ |
| Only EBV infection | 281 (23.1) | 163 (26) | 118 (20.1) | .017∗ |
| Other infection | 299 (24.6) | 108 (17.2) | 191 (32.5) | <.001∗∗∗ |
| RHD, n (%) | 195 (16) | 71 (11.3) | 124 (21.1) | < .001∗∗∗ |
| Other causes, n (%) | 61 (5) | 25 (4) | 36 (6.1) | .116 |
| Unknown, n (%) | 94 (7.7) | 53 (8.5) | 41 (7) | .391 |
| Fever, median (IQR) | 39.2 (38.8-39.9) | 39.4 (39-40) | 39.05 (38.5-39.8) | <.001∗∗∗ |
| Splenomegaly, n (%) | 674 (58.3) | 488 (82) | 186 (33.1) | <.001∗∗∗ |
| Hemophagocytosis, n (%) | 565 (60.2) | 399 (73.5) | 166 (41.9) | <.001∗∗∗ |
| Cytopenia ≥2 lineages, n (%) | 673 (55.4) | 504 (80.4) | 169 (28.7) | <.001∗∗∗ |
| Low NK-cell activity, n (%) | 316 (52.3) | 216 (59) | 100 (42) | <.001∗∗∗ |
| Elevated sCD25§, n (%) | 245 (40.7) | 210 (57.9) | 35 (14.6) | <.001∗∗∗ |
| SF >2000 μg/L, n (%) | 873 (73.8) | 486 (78.1) | 387 (69) | <.001∗∗∗ |
| FIB, median (IQR), g/L | 2.44 (1.39-3.99) | 1.57 (1.18-2.92) | 3.14 (2.2-4.74) | <.001∗∗∗ |
| TG, median (IQR), mmol/L | 2.12 (1.39-3.26) | 2.5 (1.67-3.49) | 1.78 (1.27-2.64) | <.001∗∗∗ |
| C3, median (IQR), g/L | 0.72 (0.52-0.95) | 0.63 (0.48-0.8) | 0.84 (0.61-1.06) | <.001∗∗∗ |
| C4, median (IQR), g/L | 0.21 (0.14-0.28) | 0.21 (0.13-0.29) | 0.21 (0.15-0.28) | .582 |
| No. of HLH-2004 criteria fulfilled, median (IQR) | 5 (3-5) | 5 (5-6) | 3 (2-4) | <.001∗∗∗ |
| Variables . | Total (N = 1215) . | HLH (n = 627) . | Partial HLH (n = 588) . | P value† . |
|---|---|---|---|---|
| Age, median (IQR), y | 51 (34-63) | 51 (34-63) | 52 (33-63) | .872 |
| Female, n (%) | 645 (53.1) | 309 (49.3) | 336 (57.1) | .007∗∗ |
| HLH triggers, n (%) | ||||
| Malignancy | 285 (23.5) | 207 (33) | 78 (13.3) | <.001∗∗∗ |
| HM | 228 (18.8) | 169 (27) | 59 (10) | <.001∗∗∗ |
| T-/NK-cell lymphoma | 117 (9.6) | 84 (13.4) | 33 (5.6) | <.001∗∗∗ |
| B-cell lymphoma | 78 (6.4) | 62 (9.9) | 16 (2.7) | <.001∗∗∗ |
| Hodgkin lymphoma | 6 (0.5) | 4 (0.6) | 2 (0.3) | .688 |
| Leukemia | 14 (1.2) | 12 (1.9) | 2 (0.3) | .021∗ |
| MDS | 11 (0.9) | 6 (1) | 5 (0.9) | 1 |
| Multiple myeloma | 1 (0.1) | 1 (0.2) | 0 (0) | 1 |
| POEMS syndrome | 1 (0.1) | 0 (0) | 1 (0.2) | .484 |
| Other malignancy | 57 (4.7) | 38 (6.1) | 19 (3.2) | .028∗ |
| Infection‡ | 580 (47.7) | 271 (43.2) | 309 (52.6) | .001∗∗ |
| EBV infection | 505 (41.6) | 310 (49.4) | 195 (33.2) | <.001∗∗∗ |
| EBV with malignancy | 149 (12.3) | 121 (19.3) | 28 (4.8) | <.001∗∗∗ |
| EBV with RHD | 75 (6.2) | 26 (4.1) | 49 (8.3) | .004∗∗ |
| Only EBV infection | 281 (23.1) | 163 (26) | 118 (20.1) | .017∗ |
| Other infection | 299 (24.6) | 108 (17.2) | 191 (32.5) | <.001∗∗∗ |
| RHD, n (%) | 195 (16) | 71 (11.3) | 124 (21.1) | < .001∗∗∗ |
| Other causes, n (%) | 61 (5) | 25 (4) | 36 (6.1) | .116 |
| Unknown, n (%) | 94 (7.7) | 53 (8.5) | 41 (7) | .391 |
| Fever, median (IQR) | 39.2 (38.8-39.9) | 39.4 (39-40) | 39.05 (38.5-39.8) | <.001∗∗∗ |
| Splenomegaly, n (%) | 674 (58.3) | 488 (82) | 186 (33.1) | <.001∗∗∗ |
| Hemophagocytosis, n (%) | 565 (60.2) | 399 (73.5) | 166 (41.9) | <.001∗∗∗ |
| Cytopenia ≥2 lineages, n (%) | 673 (55.4) | 504 (80.4) | 169 (28.7) | <.001∗∗∗ |
| Low NK-cell activity, n (%) | 316 (52.3) | 216 (59) | 100 (42) | <.001∗∗∗ |
| Elevated sCD25§, n (%) | 245 (40.7) | 210 (57.9) | 35 (14.6) | <.001∗∗∗ |
| SF >2000 μg/L, n (%) | 873 (73.8) | 486 (78.1) | 387 (69) | <.001∗∗∗ |
| FIB, median (IQR), g/L | 2.44 (1.39-3.99) | 1.57 (1.18-2.92) | 3.14 (2.2-4.74) | <.001∗∗∗ |
| TG, median (IQR), mmol/L | 2.12 (1.39-3.26) | 2.5 (1.67-3.49) | 1.78 (1.27-2.64) | <.001∗∗∗ |
| C3, median (IQR), g/L | 0.72 (0.52-0.95) | 0.63 (0.48-0.8) | 0.84 (0.61-1.06) | <.001∗∗∗ |
| C4, median (IQR), g/L | 0.21 (0.14-0.28) | 0.21 (0.13-0.29) | 0.21 (0.15-0.28) | .582 |
| No. of HLH-2004 criteria fulfilled, median (IQR) | 5 (3-5) | 5 (5-6) | 3 (2-4) | <.001∗∗∗ |
HM, hematologic malignancy; MDS, myelodysplastic syndromes; POEMS, polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes; SF, serum ferritin.
∗P < .05; ∗∗P < .01; ∗∗∗P < .001.
Infection means only EBV infection and other infection.
Elevated sCD25 means sCD25 ≥2400 U/mL or ≥6400 pg/mL.