Diagnostic criteria
| Criterium . | Total . | Malignancy . | Infection . | Autoimmune . | Idiopathic . | ||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| N . | Total . | (%) . | N . | Total . | (%) . | N . | Total . | (%) . | N . | Total . | (%) . | N . | Total . | (%) . | |
| Ferritin | |||||||||||||||
| Ferritin ≥ 500 μg/L | 62 | 62 | (100) | 22 | 22 | (100) | 22 | 22 | (100) | 10 | 10 | (100) | 8 | 8 | (100) |
| Ferritin ≥ 2000 μg/L | 60 | 62 | (97) | 22 | 22 | (100) | 22 | 22 | (100) | 8 | 10 | (80) | 8 | 8 | (100) |
| Ferritin > 6000 μg/L | 45 | 62 | (73) | 17 | 22 | (77) | 16 | 22 | (73) | 7 | 10 | (70) | 5 | 8 | (63) |
| Temperature | |||||||||||||||
| Fever | 60 | 62 | (97) | 22 | 22 | (100) | 21 | 22 | (95) | 10 | 10 | (100) | 7 | 8 | (88) |
| Temperature ≥ 38.4°C | 56 | 60 | (93) | 20 | 21 | (95) | 19 | 21 | (90) | 10 | 10 | (100) | 7 | 8 | (88) |
| Temperature > 39.4°C | 33 | 60 | (55) | 10 | 21 | (48) | 13 | 21 | (62) | 5 | 10 | (50) | 5 | 8 | (63) |
| Organomegaly | |||||||||||||||
| Splenomegaly | 54 | 60 | (90) | 19 | 20 | (95) | 18 | 22 | (82) | 10 | 10 | (100) | 7 | 8 | (88) |
| Hepatomegaly | 38 | 61 | (62) | 11 | 21 | (52) | 15 | 22 | (68) | 7 | 10 | (70) | 5 | 8 | (63) |
| Hepatosplenomegaly | 38 | 61 | (62) | 11 | 21 | (52) | 15 | 22 | (68) | 7 | 10 | (70) | 5 | 8 | (63) |
| Hemoglobin | |||||||||||||||
| Hemoglobin < 9.0 g/dL | 44 | 62 | (71) | 17 | 22 | (77) | 14 | 22 | (64) | 8 | 10 | (80) | 5 | 8 | (63) |
| Hemoglobin ≤ 9.2 g/dL | 47 | 62 | (76) | 20 | 22 | (91) | 14 | 22 | (64) | 8 | 10 | (80) | 5 | 8 | (63) |
| Platelets | |||||||||||||||
| Platelets < 100/nL | 49 | 62 | (79) | 19 | 22 | (86) | 19 | 22 | (86) | 4 | 10 | (40) | 7 | 8 | (88) |
| Platelets ≤ 110/nL | 52 | 62 | (84) | 20 | 22 | (91) | 19 | 22 | (86) | 6 | 10 | (60) | 7 | 8 | (88) |
| White blood cells | |||||||||||||||
| Neutrophils < 1.0/nL | 26 | 61 | (43) | 10 | 22 | (45) | 8 | 21 | (38) | 5 | 10 | (50) | 3 | 8 | (38) |
| Leukocytes ≤ 5.0/nL | 50 | 62 | (81) | 19 | 22 | (86) | 17 | 22 | (77) | 9 | 10 | (90) | 5 | 8 | (63) |
| Cytopenia | |||||||||||||||
| Bicytopenia (HLH-04) | 43 | 62 | (69) | 17 | 22 | (77) | 14 | 22 | (64) | 5 | 10 | (50) | 7 | 8 | (88) |
| Bicytopenia (HScore) | 55 | 62 | (89) | 21 | 22 | (95) | 18 | 22 | (82) | 9 | 10 | (90) | 7 | 8 | (88) |
| Pancytopenia (HLH-04) | 18 | 61 | (30) | 8 | 22 | (36) | 7 | 21 | (33) | 3 | 10 | (30) | 0 | 8 | (0) |
| Pancytopenia (HScore) | 33 | 62 | (53) | 17 | 22 | (77) | 10 | 22 | (45) | 4 | 10 | (40) | 2 | 8 | (25) |
| sCD25 ≥ 2400 U/mL | 53 | 60 | (88) | 20 | 21 | (95) | 20 | 21 | (95) | 8 | 10 | (80) | 5 | 8 | (63) |
| Histopath. hemophagocytosis | 33 | 57 | (58) | 7 | 19 | (37) | 12 | 20 | (60) | 8 | 10 | (80) | 6 | 8 | (75) |
| Triglycerides | |||||||||||||||
| TGs ≥ 132.7 mg/dL | 55 | 61 | (90) | 20 | 22 | (91) | 20 | 22 | (91) | 9 | 10 | (90) | 6 | 7 | (86) |
| TGs ≥ 265 mg/dL | 32 | 61 | (52) | 15 | 22 | (68) | 11 | 22 | (50) | 3 | 10 | (30) | 3 | 7 | (43) |
| TGs > 354 mg/dL | 20 | 61 | (33) | 8 | 22 | (36) | 8 | 22 | (36) | 3 | 10 | (30) | 1 | 7 | (14) |
| Fibrinogen | |||||||||||||||
| Fibrinogen ≤ 1.5 g/L | 29 | 61 | (48) | 13 | 21 | (62) | 9 | 22 | (41) | 3 | 10 | (30) | 4 | 8 | (50) |
| Fibrinogen ≤ 2.5 g/L | 43 | 61 | (70) | 15 | 21 | (71) | 17 | 22 | (77) | 6 | 10 | (60) | 5 | 8 | (63) |
| TGs ≥ 265 mg/dL or fibrinogen < 1.5 g/L | 43 | 61 | (70) | 18 | 21 | (86) | 15 | 22 | (68) | 5 | 10 | (50) | 5 | 8 | (63) |
| Low/absent NK cell activity | 3 | 13 | (23) | 0 | 2 | (0) | 2 | 7 | (29) | 1 | 2 | (50) | 0 | 2 | (0) |
| AST ≥ 30 U/L | 56 | 62 | (90) | 19 | 22 | (86) | 21 | 22 | (95) | 8 | 10 | (80) | 8 | 8 | (100) |
| Previous immunosuppression | 20 | 62 | (32) | 10 | 22 | (45) | 5 | 22 | (23) | 2 | 10 | (20) | 3 | 8 | (38) |
| Criterium . | Total . | Malignancy . | Infection . | Autoimmune . | Idiopathic . | ||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| N . | Total . | (%) . | N . | Total . | (%) . | N . | Total . | (%) . | N . | Total . | (%) . | N . | Total . | (%) . | |
| Ferritin | |||||||||||||||
| Ferritin ≥ 500 μg/L | 62 | 62 | (100) | 22 | 22 | (100) | 22 | 22 | (100) | 10 | 10 | (100) | 8 | 8 | (100) |
| Ferritin ≥ 2000 μg/L | 60 | 62 | (97) | 22 | 22 | (100) | 22 | 22 | (100) | 8 | 10 | (80) | 8 | 8 | (100) |
| Ferritin > 6000 μg/L | 45 | 62 | (73) | 17 | 22 | (77) | 16 | 22 | (73) | 7 | 10 | (70) | 5 | 8 | (63) |
| Temperature | |||||||||||||||
| Fever | 60 | 62 | (97) | 22 | 22 | (100) | 21 | 22 | (95) | 10 | 10 | (100) | 7 | 8 | (88) |
| Temperature ≥ 38.4°C | 56 | 60 | (93) | 20 | 21 | (95) | 19 | 21 | (90) | 10 | 10 | (100) | 7 | 8 | (88) |
| Temperature > 39.4°C | 33 | 60 | (55) | 10 | 21 | (48) | 13 | 21 | (62) | 5 | 10 | (50) | 5 | 8 | (63) |
| Organomegaly | |||||||||||||||
| Splenomegaly | 54 | 60 | (90) | 19 | 20 | (95) | 18 | 22 | (82) | 10 | 10 | (100) | 7 | 8 | (88) |
| Hepatomegaly | 38 | 61 | (62) | 11 | 21 | (52) | 15 | 22 | (68) | 7 | 10 | (70) | 5 | 8 | (63) |
| Hepatosplenomegaly | 38 | 61 | (62) | 11 | 21 | (52) | 15 | 22 | (68) | 7 | 10 | (70) | 5 | 8 | (63) |
| Hemoglobin | |||||||||||||||
| Hemoglobin < 9.0 g/dL | 44 | 62 | (71) | 17 | 22 | (77) | 14 | 22 | (64) | 8 | 10 | (80) | 5 | 8 | (63) |
| Hemoglobin ≤ 9.2 g/dL | 47 | 62 | (76) | 20 | 22 | (91) | 14 | 22 | (64) | 8 | 10 | (80) | 5 | 8 | (63) |
| Platelets | |||||||||||||||
| Platelets < 100/nL | 49 | 62 | (79) | 19 | 22 | (86) | 19 | 22 | (86) | 4 | 10 | (40) | 7 | 8 | (88) |
| Platelets ≤ 110/nL | 52 | 62 | (84) | 20 | 22 | (91) | 19 | 22 | (86) | 6 | 10 | (60) | 7 | 8 | (88) |
| White blood cells | |||||||||||||||
| Neutrophils < 1.0/nL | 26 | 61 | (43) | 10 | 22 | (45) | 8 | 21 | (38) | 5 | 10 | (50) | 3 | 8 | (38) |
| Leukocytes ≤ 5.0/nL | 50 | 62 | (81) | 19 | 22 | (86) | 17 | 22 | (77) | 9 | 10 | (90) | 5 | 8 | (63) |
| Cytopenia | |||||||||||||||
| Bicytopenia (HLH-04) | 43 | 62 | (69) | 17 | 22 | (77) | 14 | 22 | (64) | 5 | 10 | (50) | 7 | 8 | (88) |
| Bicytopenia (HScore) | 55 | 62 | (89) | 21 | 22 | (95) | 18 | 22 | (82) | 9 | 10 | (90) | 7 | 8 | (88) |
| Pancytopenia (HLH-04) | 18 | 61 | (30) | 8 | 22 | (36) | 7 | 21 | (33) | 3 | 10 | (30) | 0 | 8 | (0) |
| Pancytopenia (HScore) | 33 | 62 | (53) | 17 | 22 | (77) | 10 | 22 | (45) | 4 | 10 | (40) | 2 | 8 | (25) |
| sCD25 ≥ 2400 U/mL | 53 | 60 | (88) | 20 | 21 | (95) | 20 | 21 | (95) | 8 | 10 | (80) | 5 | 8 | (63) |
| Histopath. hemophagocytosis | 33 | 57 | (58) | 7 | 19 | (37) | 12 | 20 | (60) | 8 | 10 | (80) | 6 | 8 | (75) |
| Triglycerides | |||||||||||||||
| TGs ≥ 132.7 mg/dL | 55 | 61 | (90) | 20 | 22 | (91) | 20 | 22 | (91) | 9 | 10 | (90) | 6 | 7 | (86) |
| TGs ≥ 265 mg/dL | 32 | 61 | (52) | 15 | 22 | (68) | 11 | 22 | (50) | 3 | 10 | (30) | 3 | 7 | (43) |
| TGs > 354 mg/dL | 20 | 61 | (33) | 8 | 22 | (36) | 8 | 22 | (36) | 3 | 10 | (30) | 1 | 7 | (14) |
| Fibrinogen | |||||||||||||||
| Fibrinogen ≤ 1.5 g/L | 29 | 61 | (48) | 13 | 21 | (62) | 9 | 22 | (41) | 3 | 10 | (30) | 4 | 8 | (50) |
| Fibrinogen ≤ 2.5 g/L | 43 | 61 | (70) | 15 | 21 | (71) | 17 | 22 | (77) | 6 | 10 | (60) | 5 | 8 | (63) |
| TGs ≥ 265 mg/dL or fibrinogen < 1.5 g/L | 43 | 61 | (70) | 18 | 21 | (86) | 15 | 22 | (68) | 5 | 10 | (50) | 5 | 8 | (63) |
| Low/absent NK cell activity | 3 | 13 | (23) | 0 | 2 | (0) | 2 | 7 | (29) | 1 | 2 | (50) | 0 | 2 | (0) |
| AST ≥ 30 U/L | 56 | 62 | (90) | 19 | 22 | (86) | 21 | 22 | (95) | 8 | 10 | (80) | 8 | 8 | (100) |
| Previous immunosuppression | 20 | 62 | (32) | 10 | 22 | (45) | 5 | 22 | (23) | 2 | 10 | (20) | 3 | 8 | (38) |
The fulfilled diagnostic criteria of the HLH-2004 criteria and the HScore of the total population and within the etiologic subgroups is shown.
AST, aspartate transferase; Histopath., histopathological; TG, triglyceride.