Sézary Syndrome with Erythroderma and High Blood Tumour Burden (T4 N2/3/x M0 B2)
This protocol covers mycosis fungoides presenting as Sézary syndrome staged at T4 N2/3/x M0 B2 — defined by erythroderma and a high blood tumour burden.
Clinical Scenario
Sézary syndrome staged as T4 N2/3/x M0 B2, with erythroderma and high blood tumour burden. This staging places the disease in an advanced systemic category requiring protocol-directed treatment.
Treatment Approach
The protocol identifies second-line systemic therapy as the recommended approach, with several options considered clinically equivalent. The specific agents, selection criteria, and sequencing algorithm are set out in the full structured protocol.
References
DOI: 10.1016/j.ejca.2023.113343
- SS is staged as T4 N2/3/x M0 B2.
- The drug is approved in Europe for the treatment of adult patients with MF or SS who have received at least one prior systemic therapy.
- Based on the results of the ALCANZA trial (see below) the drug is approved in Europe for the treatment of adult patients with CD30+ cutaneous T-cell lymphoma (CTCL) after at least one prior systemic therapy.
- A systematic review from 2018 confirmed earlier findings of its efficacy in SS and of reduced toxicity with low dose regimens.
- Gemcitabine is another cytostatic drug recommended for the treatment of advanced stage MF and SS.
- Cyclophosphamide Hydroxydaunorubicin (Doxorubicin) Oncovin (Vincristin) Prednisone (CHOP) is the most widely used regimen with a number of variants and other combinations available.
- Apart from the rare situation of local radiotherapy for mono-localised MF (see above) allogeneic stem cell transplantation (alloSCT) is the only option in MF/SS with curative intention in patients with advanced disease.
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