Clinical and laboratory features of patients with EBV-HLH
| . | Patient . | |||
|---|---|---|---|---|
| EBV-HLH1 . | EBV-HLH2 . | EBV-HLH3 . | EBV-HLH4 . | |
| Age, y/sex | 1/M | 5/F | 2/F | 1/M |
| Fever, d (> 38.5°C) | 23 | 15 | 20 | 21 |
| Hepatosplenomegaly (cm) | 4/1 | 3/− | 6/3 | 2/− |
| Lymphoadenopathy | + | + | + | + |
| Jaundice | + | + | + | + |
| Coagulopathy | − | + | + | + |
| CNS symptom | − | + | + | − |
| Blood leukocyte (/mm3) | 500 | 4 500 | 1 300 | 1 200 |
| Platelets (× 104/mm3) | 2.4 | 5.8 | 5.1 | 3.6 |
| Serum ferritin (mg/mL) | 38 000 | 122 000 | 7 733 | 55 398 |
| Serum LDH (IU/mL) | 9 190 | 20 270 | 4 765 | 19 240 |
| Urinary β2-microglobulin (μg/mL) | 45 000 | 28 623 | 43 610 | ND |
| Treatment | ET, pulse | PE, pulse | PE, pulse | ET, pulse |
| CSA, VP-16 | CSA | CSA | CSA, VP-16 | |
| Anti-EBV antibody titer to | ||||
| VCA-IgG | 2 560 | 640 | 1 280 | 320 |
| VCA-IgM | < 10 | < 10 | < 10 | < 10 |
| EA-DR IgG | < 10 | 10 | < 10 | < 10 |
| EBNA | 10 | < 10 | < 10 | < 10 |
| Outcome | Alive | Deceased | Alive | Alive |
| . | Patient . | |||
|---|---|---|---|---|
| EBV-HLH1 . | EBV-HLH2 . | EBV-HLH3 . | EBV-HLH4 . | |
| Age, y/sex | 1/M | 5/F | 2/F | 1/M |
| Fever, d (> 38.5°C) | 23 | 15 | 20 | 21 |
| Hepatosplenomegaly (cm) | 4/1 | 3/− | 6/3 | 2/− |
| Lymphoadenopathy | + | + | + | + |
| Jaundice | + | + | + | + |
| Coagulopathy | − | + | + | + |
| CNS symptom | − | + | + | − |
| Blood leukocyte (/mm3) | 500 | 4 500 | 1 300 | 1 200 |
| Platelets (× 104/mm3) | 2.4 | 5.8 | 5.1 | 3.6 |
| Serum ferritin (mg/mL) | 38 000 | 122 000 | 7 733 | 55 398 |
| Serum LDH (IU/mL) | 9 190 | 20 270 | 4 765 | 19 240 |
| Urinary β2-microglobulin (μg/mL) | 45 000 | 28 623 | 43 610 | ND |
| Treatment | ET, pulse | PE, pulse | PE, pulse | ET, pulse |
| CSA, VP-16 | CSA | CSA | CSA, VP-16 | |
| Anti-EBV antibody titer to | ||||
| VCA-IgG | 2 560 | 640 | 1 280 | 320 |
| VCA-IgM | < 10 | < 10 | < 10 | < 10 |
| EA-DR IgG | < 10 | 10 | < 10 | < 10 |
| EBNA | 10 | < 10 | < 10 | < 10 |
| Outcome | Alive | Deceased | Alive | Alive |
ND indicates not determined; ET, exchange transfusion; pulse, methylprednisolone pulse therapy; PE, plasma exchange; CSA, cyclosporin A; VP-16, etoposide; VCA, viral capsid antigen; EA, early antigen; EBNA, EBV-associated nuclear antigen.