TABLE I.
Clinical Characteristics and Outcome of Pediatric Patients With SPTCL
| Patient | Age(years, at biopsy), sex |
Extent cutaneous lesions (at presentation) |
Systemic symptoms | Cytopenia(s) | HSM, LAD | Treatment | Recurrence | Last known |
|---|---|---|---|---|---|---|---|---|
| 1 | 9, F | S, T | ND | ND | ND | IS (prednisone, tacrolimus, cyclosporine) | Yes | A, NED (139 mo) |
| 2 | 14, F | S, T | ND | ND | ND | Asparaginase, MTX, adriamycin, vincristine, carmustine, prednisone | No | D, other cause |
| 3 | 3, F | Mul, ND | ND | Pancytopenia | Shotty LAD | Unk | Yesa | Unk |
| 4 | 19, F | Mul, ND | ND | ND | ND | CHOP x 6 cycles | Yes | Unk |
| 5 | 21, F | S, T | Fever, fatigue, weight loss | Anemia, leukopenia | HSM, right axillary LAD | Prior cyclosporine | Yesa | A, NED (> 17 years) |
| 6 | 7, F | S, T | ND | ND | ND | Prior CHOP | Yesa | Unk |
| 7 | 3, F | S, E | ND | ND | ND | CHOP, maintenance with bexarotene | No | A, NED (24+ mo) |
| 8 | 6, F | M, E | ND | ND | HSM | Unk | Unk | |
| 9 | 18, M | Mul, T/E | Recent flu-like illness also in family members | Mild anemia, leukopenia | CT negative | Unk | No | A, NED (38 mo) |
| 10 | 5 Mo, F | Mul, T/E | None | Mild anemia | CT negative | Unk | Unk | Unk |
| 11 | 18, F | Mul, T/E | High fever | Leukopenia (lymphopenia), anemia | CT-no LAD, borderline splenomegaly | Unk | Unk | Unk |
| 12 | 4, M | Mul, E | ND | ND | ND | Steroids alone | Unk | Unk |
| 13 | 20, M | S, T | None | Anemia, leukopenia (neutropenia) | CT- mild splenomegaly, small left axillary LAD | Unk | No | A, NED (28 mo) |
| 14 | 5 Mo, M | Mul, T | ND | ND | ND | Unk | Unk | Unk |
| 15 | 16 Mo, M | Mul, T/E | Mild fever | None | ND | None | Unk | A (2 mo), lost to follow-up |
| 16 | 21, M | Mul, T & head | None | Intermittent leukopenia (for many years) | PET/CT-small retroperitoneal LAD | Unk | Unk | Unk |
Mo, months; F, female; M, male; S, single; Mul, multiple; T, trank; E, extremities; ND, not described; HSM, hepatosplenomegaly; LAD, lymphadenopathy; IS, immunosuppressives; MTX, methotrexate; CHOP, cyclophosphamide, doxorubicin, vincristine, prednisolone; Unk, unknown; A, alive; NED, no evidence of disease; D, deceased, indicates cases in which the recurrent but not original diagnostic specimen was reviewed.