Table 1.
Characteristics of five elderly patients and eight children and young adult patients
| No. | Sex | Age (years) | Onset (months) | Associated disease | Treatment | Follow-up (months) | Outcome | EBV serology | EBV DNA in PBa | Histologic grade (necrosis) | Cell type | TCR gene rearrangement |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Elderly patients (n = 5) | ||||||||||||
| 1 | M | 50 | 1b | HBV carrierc | CHOP | 7 | Dead | Anti-VCA IgG+, IgM−, EA−, EBNA+ | ∼30,530 | 3 | αβ-T CD8>CD4, CD56− | Monoclonal |
| 2 | M | 51 | 1 | HBV carrierc | CHOP, A-DHAP, autoPBSCT | 14 | Dead | Anti-VCA IgG+, IgM−, EA−, EBNA+ | ∼187 | 3 | αβ-T CD8>CD4 CD56− | Monoclonal |
| 3 | M | 60 | <1 | None | CHOP | 5 | Dead | Anti-VCA IgG+, IgM−, EA±, EBNA+ | ∼14,770 | 1(+) | γδ-Td, CD4−, CD8-CD56− | Monoclonal |
| 4 | F | 64 | <1 | Hepatitis Cc | Cytoxan, VCR | 1 | Dead | NA | NA | 2 | NA CD56− | NA |
| 5 | M | 84 | <1 | Prostate ca | Cytoxan, VCR | 3 | Dead | NA | NA | 2 | αβ-T CD8+, CD56− | Monoclonal |
| Children and young adults (n = 8) | ||||||||||||
| 6 | M | 10 | 24 | CAEBV | 106B, VCR, TMX | 10 | Dead | Anti-VCA IgG+, IgM−, EA+, EBNA− | ∼14,680 | 1 | αβ-T CD4>CD8 CD56− | Monoclonal |
| 7 | M | 11 | <1 | None | 106B | 1 | Dead | Anti-VCA IgG+, IgM−, EA−, EBNA+ | NA | 2 | αβ-Te CD8+CD56− | Monoclonal |
| 8 | M | 11 | <1 | None | Steroid | 0.3 | Dead | NA | 19,220 | 2(+) | αβ-T CD8+ | Monoclonal |
| 9 | F | 14 | <1 | None | 1 | Dead | Anti-VCA IgG+, IgM−, EBNA− | NA | 3 | NA CD56− | NA | |
| 10 | F | 15 | 12 | Hydroaf vacciniforme | CHOP, ESHAP | 6 | Dead | NA | NA | 2 | αβ-T CD8+, CD56− | NA |
| 11 | M | 16 | <1 | None | CHOP, DHAP, IMVP-16/PD, EPHOCH | 6 | Dead | NA | NA | 3 | αβ-T CD8+, CD56− | NA |
| 12 | F | 20 | 6 | CAEBV | CHOP, IMVP-16/PD, DHAP, ICE | 12 | Dead | Anti-VCA IgG+, IgM−, EA+ EBNA± | NA | 1 | αβ-T CD8>CD4 CD56− | Monoclonal |
| 13 | F | 34 | 12 | CAEBV | CHOP | 3 | Dead | Anti-VCA IgG+, IgM−, EA± EBNA− | NA | 2(+) | NA | Monoclonal |
106B Prednisolone+cyclophosphamide+daunorubicin+vincristine+l-asparaginase, VCR vincristine, TMX trimethoprim-sulfamethoxazol, CHOP cyclophosphamide+adriamycin+vincristine+prednisolone, DHAP dexametasone+cytarabine+cisplatin, IMVP-16/PD ifosfamide+methotrexate+VP-16 (etoposide)+prednisolone, EPHOCH etoposide+cytoxan+doxorubicin+vincristine+prednisolone, ESHAP, etoposide+methylprednisolone+cytarabine+cisplatin, ICE, ifosfamide+carboplatin+etoposide, NA not available
aCopies/reaction (5 μl) using whole blood
bEnlarged lymph node at infraauricular area at 45 years old, followed by decreased size
cThe patients had no treatment for hepatitis.
dThis case was regarded of γ/δ phenotype due to the lack of staining with the βF1 antibody in the proper reactivity with internal control.
eDetermined by flow cytometry
fHydroa vacciniform-like skin lesion with photosensitivity on face and arm for many years