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.