No RCTs were reported. A systematic review (1987-98) of response rates and outcomes in open non-randomised and mostly retrospective studies of extracorporeal photopheresis (ECP) in erythrodermic CTCL (stage III/IVA) showed an OR rate of 35-71%, with CR rates of 14-26%.42 Responses have been assessed mostly using a similar scoring system to that devised for the original study.43
A further retrospective study of 34 patients with mostly erythrodermic CTCL (22 stage IV, 10 stage III, and 2 stage I) reported an OR rate of 50%, with 18% achieving CR.44 Response was restricted to those with erythrodermic disease. This study involved a modified "accelerated" treatment schedule consisting of nine (as opposed to six) collections during each cycle and an increase to twice-monthly treatment if there was a lack of response.
Other studies have reported minor responses (25-50% improvement) but this would not satisfy accepted criteria for PR. ECP is generally administered on two consecutive days (one cycle) each month and it is accepted that at least six cycles are required to assess response. Survival data have been reported in four studies of erythrodermic disease, with median survivals of 39-100 months from diagnosis.45-48
A randomised crossover study comparing ECP with PUVA in patients with non-erythrodermic (stage IB-IIA) mycosis fungoides has shown no clinical efficacy for ECP in early stages of the disease compared with PUVA.49 In contrast, other uncontrolled studies have reported successful responses in patients with non-erythrodermic disease.
A 9-year retrospective study of ECP alone in 37 patients (68% stage IB, 5% stage IIB, 27% stage III) showed a CR rate of 14% and a PR rate of 41%, with an improved response rate in resistant patients with the addition of alfa interferon.50
A prospective open non-randomised study of 14 patients with non-erythrodermic mycosis fungoides (stage IIA/IIB) treated with combined alfa interferon (maximum tolerated dose of 18 MU three times weekly) and ECP for 6 months showed a CR in four and a PR in three (OR 50%) but this design does not exclude responses to alfa interferon alone.51
A non-randomised retrospective study in erythrodermic disease (stage III/IVA) (19911996) showed that six of nine patients treated with alfa interferon plus ECP showed a response (with four CRs) while only one of 10 patients treated with ECP alone achieved a CR. In the patients achieving a CR, lymph node disease also resolved.52 In contrast, the combination of alfa interferon plus ECP failed to produce significant clinical responses in six patients with Sezary syndrome,53 although isolated case reports have described patients with Sezary syndrome in whom a complete clinical and molecular remission has been achieved with this combination.54,55
A retrospective non-randomised study (1974-97) has compared DSS and OS in 44 patients with erythrodermic CTCL treated with either TSEB alone or TSEB and adjuvant or neoadjuvant ECP (see below).
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