Efficacy

An RCT in 103 CTCL patients comparing TSEB and multiagent chemotherapy (CAVE) with sequential topical therapy including superficial radiotherapy and phototherapy revealed a higher CR rate in the TSEB/chemotherapy group (38% compared with 18%; P= 0-032 with OR rates of 90% and 65%, respectively) but after a median follow up of 75 months there was no significant difference in DFS or OS.77

A systematic review of all systemic therapy in CTCL (mycoses fungoides and Sezary syndrome, 1988-94) showed an OR rate of 81% in 331 patients treated with various different combination chemotherapeutic regimens, with a CR rate of 38% and response duration ranging from 5 to 41 months, with no documented cures for patients with late stages of disease (IIB-IVB).59

Recent prospective non-randomised studies of different multiagent chemotherapy regimens have revealed similar OR rates. A third-generation anthracycline (idarubicin) was used in combination with etoposide, cyclophosphamide, vincristine, prednisolone and bleomycin (VICOP-B) to treat 25 CTCL patients (eight stage IIB, 13 IVA, four stage IVB) for 12 weeks. OR rates of 80%, with 36% CR, were documented, although

10 patients had not received any previous therapy. The two patients with Sezary syndrome did not respond and the median duration of response in patients with mycoses fungoides was 8-7 months. Stage IIB patients had a median duration of response of 22 months but four previously untreated patients received additional TSEB therapy after completion of chemotherapy.87

A combination of etoposide, vincristine, doxorubicin, cyclophosphamide and prednisolone (EPOCH) was used to treat 15 patients with advanced, refractory CTCL (six with Sezary syndrome; four with stage-IVB mycoses fungoides, one with adult T-cell lymphoma and four with large cell anaplastic lymphoma). After a median of five cycles, 27% had a CR and 53% achieved a PR (OR rate 80%) with an overall median survival of 13-5 months.88

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