Treatment of Sézary Syndrome Refractory or with Contraindications to First-Line Therapy
Sézary syndrome is an aggressive cutaneous T-cell lymphoma with a challenging clinical course. When first-line therapy does not produce an adequate response — or when it cannot be used — a structured approach to second-line systemic management is required.
Clinical scenario
This protocol applies to patients with Sézary syndrome who are refractory to first-line treatment or for whom first-line therapy carries contraindications that preclude its use.
Treatment approach (partial overview)
In this setting, second-line options include targeted monoclonal antibody-based therapies and, in carefully selected patients with advanced disease and poor prognosis, more intensive systemic interventions.
Full eligibility criteria, regimen details, and sequencing are available in the complete protocol.
References
DOI: 10.1016/j.ejca.2023.113343
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.
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.
AlloSCT should be considered only in patients with advanced disease and poor prognosis but without significant comorbidity.
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