Treatment of Mycosis Fungoides in Sézary Syndrome with Erythroderma and High Blood Tumour Burden

Clinical Scenario

This protocol covers Mycosis fungoides presenting as Sézary syndrome — staged T4 N2/3/x M0 B2, with erythroderma and a high blood tumour burden. This is a first-line treatment setting.

Sézary Syndrome

Sézary syndrome is staged as T4 N2/3/x M0 B2. This staging defines the specific sub-population — characterised by erythroderma (T4) and high circulating tumour burden (B2) — for which this first-line protocol applies.

Treatment Approach (Partial Overview)

First-line management includes extracorporeal photochemotherapy (ECP), which may be used alone or alongside systemic agents. Several options are considered equivalent at the first-line stage — the complete regimen, including all recognised combinations and the criteria guiding their selection, is available in the full protocol.

Instant Access to Structured Evidence-Based Regimens

References

SS is staged as T4 N2/3/x M0 B2.
It thus still applies that ECP can be safely applied alone or in combination with other systemic and skin directed therapies and its use is recommended mainly in erythrodermic MF and SS without high level evidence on superiority of one combination over the other or over ECP alone.

DOI: 10.1016/j.ejca.2023.113343

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