Sézary syndrome ICD-10 C84.1 · ICD-11 2B02

First-Line Management of Sézary Syndrome

Sézary syndrome requires a structured first-line approach. Several initial treatment options are currently considered equivalent, making evidence-based guidance essential for selecting and sequencing therapy appropriately.

First-Line Treatment Approach

Management is anchored in extracorporeal photochemotherapy (ECP), which may be used as a standalone strategy or in combination with systemic agents. Multiple first-line regimens are recognised as equivalent options for erythrodermic presentations. The complete set of alternatives, combination strategies, and clinical considerations extend well beyond this summary.

Full regimen, options, and clinical decision points are in the complete protocol ↓

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1016/j.ejca.2023.113343

ECP can be used alone or in combination with skin directed and other systemic therapies.
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.

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