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Patients with late stages of CTCL (IIB-IVB) will require treatment with a chemotherapy regimen, although response duration is short. In the RCT,77 OS/DFS was similar to that in those treated with skin-directed therapy although in this study patients with early stage disease were also included. The individual patient's quality of life should always be considered before embarking on very toxic regimens with limited efficacy. Single-agent regimens (see above) appear to have similar efficacy, although studies involving a comparison between single-agent and multi-agent regimens, with or without TSEB, are required. To date there have been no studies assessing the use of biochemotherapy in CTCL although subsequent treatment with immunotherapy for patients achieving a response with chemotherapy should be considered.

Myeloablative chemotherapy with autologous/allogeneic peripheral blood/bone-marrow stem-cell transplantation Efficacy

No systematic reviews or RCTs were identified. Most studies were based on small numbers of patients. High-dose chemotherapy with TSEB and total-body irradiation (TBI) in four and three patients with mycoses fungoides (two patients had both TSEB and TBI) followed by autologous bone marrow transplantation in six patients (three stage IIB, one stage IVA, two stage IVB) produced five complete clinical responses but disease relapse occurred within 100 days in three patients.89 The other two patients, who had both received a combination of carmustine-etoposide-cisplatin chemotherapy, were disease free at almost 2 years (666 and 631 days post-transplant).

High-dose chemotherapy combined with either TSEB or TBI and followed by autologous peripheral blood stem cell transplantation in patients with stage IIB/IVA mycosis fungoides revealed CRs in eight patients and durable clinical responses in four patients (median DFS 11 months).90

Isolated case reports of high-dose chemotherapy with TBI followed by allogeneic bone-marrow or stem-cell transplantation have shown excellent long-term complete remissions in stage IIB mycosis fungoides (CR for total of 17 months at time of report) and Sezary syndrome.91,92

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