Characteristics of 30 patients with chronic active Epstein-Barr virus infection
| No. . | Sex . | Age (y) . | Onset (y) . | Outcome . | EBV-related antibodies . | EBV DNA (log) . | Treatment . | Clonality . | Cell type . | |||
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| VCA IgG . | EA IgG . | EBNA . | PBMC (copy/μg DNA) . | Plasma (copy/mL) . | ||||||||
| 1 | M | 6 | 4 | Alive | 5 120 | 2 560 | < 10 | 3.5 | 3.0 | None | n.d. | T |
| 2 | M | 6 | 1 | Alive | 320 | 160 | < 10 | 4.2 | 3.5 | ACV, ara-A, IFNα, IL2 | n.d. | T |
| 3 | M | 6 | 3 | Dead | 5 120 | 2 560 | 80 | 3.6 | 2.8 | ACV, ara-A, IFNα, CTL | mono | T(CD8) |
| 4 | M | 7 | 6 | Dead | 5 120 | 1 280 | 40 | 4.2 | 2.8 | ACV, PSL | n.d. | T |
| 5 | F | 9 | 8 | Alive | 5 120 | 640 | 40 | 3.6 | 3.0 | None | poly | T |
| 6 | M | 10 | 8 | Dead | 640 | 640 | < 10 | 3.8 | 3.6 | ara-A, PSL, VP16/PSL/CSA | n.d. | T(CD4) |
| 7 | M | 10 | 5 | Alive | 1 280 | 20 | 40 | 4.9 | n.d. | CHOP, HSCT | mono | T |
| 8 | M | 11 | 9 | Dead | 2 560 | 5 120 | 40 | 4.3 | 2.9 | ACV, GCV, IFNα, IL2, PSL | mono | T |
| 9 | F | 12 | 10 | Alive | 640 | 160 | 40 | 5.0 | 4.5 | ACV, IFNα, IL2, PSL, CHOP | oligo | T(CD4) |
| 10 | M | 12 | 7 | Alive | 5 120 | 2 560 | 40 | 3.5 | n.d. | CHOP, HSCT | mono | T |
| 11 | F | 13 | 11 | Dead | 5 120 | 1 280 | 40 | 4.2 | 3.1 | VP16/PSL/CSA, CHOP | poly | T |
| 12 | M | 18 | 13 | Alive | 1 280 | 640 | 20 | 4.5 | 2.0 | ACV, IFNα, PSL, VP16, MOPP | n.d. | T(CD8) |
| 13 | M | 18 | 11 | Alive | 10 240 | 10 240 | 10 | 4.0 | 3.6 | MOPP | n.d. | T |
| 14 | F | 19 | 14 | Dead | 1 280 | 160 | 40 | 4.8 | 4.2 | PSL, CSA, CHOP, HSCT | mono | T |
| 15 | F | 25 | 23 | Alive | > 10 240 | 2 560 | 10 | 3.9 | 2.0 | VP16/PSL/CSA | mono | T(CD4) |
| 16 | M | 29 | 27 | Alive | 2 560 | 2 560 | 20 | 3.6 | Negative | VP16/PSL/CSA | n.d. | T |
| 17 | M | 5 | 3 | Alive | 1 280 | 40 | 80 | 4.8 | 3.6 | ACV, PSL, VP16/PSL/CSA | mono | NK |
| 18 | F | 7 | 6 | Alive | 320 | 20 | 40 | 4.6 | 4.2 | None | mono | NK |
| 19 | F | 12 | 4 | Alive | 640 | 2 560 | 160 | 4.0 | n.d. | ara-A | mono | NK |
| 20 | M | 13 | 9 | Alive | 5 120 | 1 280 | 40 | 4.3 | Negative | ACV, ara-A, PSL, VP16/PSL/CSA | n.d. | NK |
| 21 | F | 14 | 2 | Dead | 2 560 | 320 | < 10 | 4.8 | 4.2 | ACV, ara-A, PSL, HSCT | mono | NK |
| 22 | F | 14 | 9 | Dead | 320 | 640 | 40 | 4.7 | n.d. | PSL, splenectomy | mono | NK |
| 23 | M | 15 | 9 | Alive | 40 | 10 | 80 | 4.6 | 4.1 | PSL | mono | NK |
| 24 | M | 16 | 2 | Alive | 80 | 40 | 10 | 4.4 | 4.6 | ara-A | mono | NK |
| 25 | M | 17 | 12 | Alive | 640 | n.d. | 10 | 3.8 | Negative | VP16/PSL/CSA, HDCA, HSCT | mono | NK |
| 26 | M | 18 | 8 | Dead | 160 | 10 | < 10 | 4.7 | Negative | VP16/PSL/CSA, HDCA, HSCT | mono | NK |
| 27 | F | 19 | 13 | Alive | 10 240 | 5 120 | 160 | 4.5 | 3.1 | None | oligo | NK |
| 28 | F | 19 | 6 | Alive | 40 | 10 | < 10 | 4.6 | Negative | PSL | n.d. | NK |
| 29 | M | 14 | 2 | Alive | 1 280 | 320 | < 10 | 2.6 | Negative | PSL | n.d. | Unclassified |
| 30 | F | 31 | 3 | Dead | 640 | 20 | 40 | 4.8 | 4.6 | PSL, VP16/PSL/CSA, HSCT | n.d. | Unclassified |
| No. . | Sex . | Age (y) . | Onset (y) . | Outcome . | EBV-related antibodies . | EBV DNA (log) . | Treatment . | Clonality . | Cell type . | |||
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| VCA IgG . | EA IgG . | EBNA . | PBMC (copy/μg DNA) . | Plasma (copy/mL) . | ||||||||
| 1 | M | 6 | 4 | Alive | 5 120 | 2 560 | < 10 | 3.5 | 3.0 | None | n.d. | T |
| 2 | M | 6 | 1 | Alive | 320 | 160 | < 10 | 4.2 | 3.5 | ACV, ara-A, IFNα, IL2 | n.d. | T |
| 3 | M | 6 | 3 | Dead | 5 120 | 2 560 | 80 | 3.6 | 2.8 | ACV, ara-A, IFNα, CTL | mono | T(CD8) |
| 4 | M | 7 | 6 | Dead | 5 120 | 1 280 | 40 | 4.2 | 2.8 | ACV, PSL | n.d. | T |
| 5 | F | 9 | 8 | Alive | 5 120 | 640 | 40 | 3.6 | 3.0 | None | poly | T |
| 6 | M | 10 | 8 | Dead | 640 | 640 | < 10 | 3.8 | 3.6 | ara-A, PSL, VP16/PSL/CSA | n.d. | T(CD4) |
| 7 | M | 10 | 5 | Alive | 1 280 | 20 | 40 | 4.9 | n.d. | CHOP, HSCT | mono | T |
| 8 | M | 11 | 9 | Dead | 2 560 | 5 120 | 40 | 4.3 | 2.9 | ACV, GCV, IFNα, IL2, PSL | mono | T |
| 9 | F | 12 | 10 | Alive | 640 | 160 | 40 | 5.0 | 4.5 | ACV, IFNα, IL2, PSL, CHOP | oligo | T(CD4) |
| 10 | M | 12 | 7 | Alive | 5 120 | 2 560 | 40 | 3.5 | n.d. | CHOP, HSCT | mono | T |
| 11 | F | 13 | 11 | Dead | 5 120 | 1 280 | 40 | 4.2 | 3.1 | VP16/PSL/CSA, CHOP | poly | T |
| 12 | M | 18 | 13 | Alive | 1 280 | 640 | 20 | 4.5 | 2.0 | ACV, IFNα, PSL, VP16, MOPP | n.d. | T(CD8) |
| 13 | M | 18 | 11 | Alive | 10 240 | 10 240 | 10 | 4.0 | 3.6 | MOPP | n.d. | T |
| 14 | F | 19 | 14 | Dead | 1 280 | 160 | 40 | 4.8 | 4.2 | PSL, CSA, CHOP, HSCT | mono | T |
| 15 | F | 25 | 23 | Alive | > 10 240 | 2 560 | 10 | 3.9 | 2.0 | VP16/PSL/CSA | mono | T(CD4) |
| 16 | M | 29 | 27 | Alive | 2 560 | 2 560 | 20 | 3.6 | Negative | VP16/PSL/CSA | n.d. | T |
| 17 | M | 5 | 3 | Alive | 1 280 | 40 | 80 | 4.8 | 3.6 | ACV, PSL, VP16/PSL/CSA | mono | NK |
| 18 | F | 7 | 6 | Alive | 320 | 20 | 40 | 4.6 | 4.2 | None | mono | NK |
| 19 | F | 12 | 4 | Alive | 640 | 2 560 | 160 | 4.0 | n.d. | ara-A | mono | NK |
| 20 | M | 13 | 9 | Alive | 5 120 | 1 280 | 40 | 4.3 | Negative | ACV, ara-A, PSL, VP16/PSL/CSA | n.d. | NK |
| 21 | F | 14 | 2 | Dead | 2 560 | 320 | < 10 | 4.8 | 4.2 | ACV, ara-A, PSL, HSCT | mono | NK |
| 22 | F | 14 | 9 | Dead | 320 | 640 | 40 | 4.7 | n.d. | PSL, splenectomy | mono | NK |
| 23 | M | 15 | 9 | Alive | 40 | 10 | 80 | 4.6 | 4.1 | PSL | mono | NK |
| 24 | M | 16 | 2 | Alive | 80 | 40 | 10 | 4.4 | 4.6 | ara-A | mono | NK |
| 25 | M | 17 | 12 | Alive | 640 | n.d. | 10 | 3.8 | Negative | VP16/PSL/CSA, HDCA, HSCT | mono | NK |
| 26 | M | 18 | 8 | Dead | 160 | 10 | < 10 | 4.7 | Negative | VP16/PSL/CSA, HDCA, HSCT | mono | NK |
| 27 | F | 19 | 13 | Alive | 10 240 | 5 120 | 160 | 4.5 | 3.1 | None | oligo | NK |
| 28 | F | 19 | 6 | Alive | 40 | 10 | < 10 | 4.6 | Negative | PSL | n.d. | NK |
| 29 | M | 14 | 2 | Alive | 1 280 | 320 | < 10 | 2.6 | Negative | PSL | n.d. | Unclassified |
| 30 | F | 31 | 3 | Dead | 640 | 20 | 40 | 4.8 | 4.6 | PSL, VP16/PSL/CSA, HSCT | n.d. | Unclassified |
VCA indicates viral capsid antigens; EA, early antigens; EBNA, EB nuclear antigens; PBMC, peripheral blood mononuclear cells; ACV, acyclovir; ara-A, vidarabine; IFNα, interferon-α; IL2, interleukin 2; CTL, cytotoxic T lymphocytes therapy; PSL, prednisolone; VP16, etoposide; CSA, cyclosporin A; CHOP, cyclophosphamide, daunorubicin, vincristine, prednisolone; HSCT, hematopoietic stem cell transplantation; MOPP, mechlorethamine, vincristine, procarbazine, prednisolone; HDCA, high-dose cytarabine; n.d., not done; NK, natural killer.