Patient characteristics and clinical findings
| . | Total cases (N = 100), n (%) . |
|---|---|
| Male/female, n | 53/47 |
| Age at diagnosis, range (median), y | 1-78 (21) |
| EBV-infected cell type | |
| CD4 | 25 (25) |
| CD8 | 13 (13) |
| CD56 | 28 (28) |
| γδT | 3 (3) |
| CD56− NK | 2 (2) |
| CD4+CD8 | 6 (6) |
| CD4+CD56 | 4 (4) |
| CD8+CD56 | 1 (1) |
| CD4+CD8+CD56 | 6 (6) |
| Others | 7 (7) |
| NA | 5 (5)* |
| Clonality of the infected cells | |
| Monoclonal | 54 (54) |
| Oligoclonal | 7 (7) |
| No clonality | 4 (4) |
| NA | 35 (35) |
| Symptoms and signs at diagnosis | |
| Fever | 85 (85) |
| Hepatosplenomegaly | 64 (70) |
| Lymphadenopathy | 48 (53) |
| Cardiac dysfunction | 8 (9) |
| Aneurysm | 8 (9) |
| Gastrointestinal symptom | 7 (8) |
| Neurological symptoms | 7 (8) |
| Vasculitis | 6 (7) |
| Uveitis | 4 (4) |
| HLH | 24 (26) |
| HMB | 25 (25) |
| HV-like eruption | 9 (9) |
| Laboratory findings | |
| Neutropenia (<1000/µL) | 14 (16) |
| Anemia (<9 g/dL) | 12 (14) |
| Thrombocytopenia (<10 × 104/µL) | 27 (30) |
| High ALT (>128 U/L) | 27 (31) |
| High sIL-2R (>2400 U/mL) | 17 (27) |
| . | Total cases (N = 100), n (%) . |
|---|---|
| Male/female, n | 53/47 |
| Age at diagnosis, range (median), y | 1-78 (21) |
| EBV-infected cell type | |
| CD4 | 25 (25) |
| CD8 | 13 (13) |
| CD56 | 28 (28) |
| γδT | 3 (3) |
| CD56− NK | 2 (2) |
| CD4+CD8 | 6 (6) |
| CD4+CD56 | 4 (4) |
| CD8+CD56 | 1 (1) |
| CD4+CD8+CD56 | 6 (6) |
| Others | 7 (7) |
| NA | 5 (5)* |
| Clonality of the infected cells | |
| Monoclonal | 54 (54) |
| Oligoclonal | 7 (7) |
| No clonality | 4 (4) |
| NA | 35 (35) |
| Symptoms and signs at diagnosis | |
| Fever | 85 (85) |
| Hepatosplenomegaly | 64 (70) |
| Lymphadenopathy | 48 (53) |
| Cardiac dysfunction | 8 (9) |
| Aneurysm | 8 (9) |
| Gastrointestinal symptom | 7 (8) |
| Neurological symptoms | 7 (8) |
| Vasculitis | 6 (7) |
| Uveitis | 4 (4) |
| HLH | 24 (26) |
| HMB | 25 (25) |
| HV-like eruption | 9 (9) |
| Laboratory findings | |
| Neutropenia (<1000/µL) | 14 (16) |
| Anemia (<9 g/dL) | 12 (14) |
| Thrombocytopenia (<10 × 104/µL) | 27 (30) |
| High ALT (>128 U/L) | 27 (31) |
| High sIL-2R (>2400 U/mL) | 17 (27) |
NA, not available.
One and 4 patients were described to be CD3 and NK infected, respectively; detailed phenotypes were not available.