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Sézary Syndrome — SCE Dermatology MCQ

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HardSkin CancerSézary SyndromeSCE Dermatology

A 63-year-old woman presents with intensely itchy erythroderma affecting more than 90% of her body surface area. She has peripheral lymphadenopathy and blood film shows atypical cerebriform lymphocytes (Sézary cells) >1000/μL. What is the most likely diagnosis?

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Correct answer: A — Sézary syndrome

Sézary syndrome is the leukaemic variant of cutaneous T-cell lymphoma, defined by the triad of erythroderma, lymphadenopathy, and circulating Sézary cells (atypical cerebriform T lymphocytes) >1000/μL in peripheral blood. It is classified as stage IVA1 or higher mycosis fungoides/Sézary syndrome. Flow cytometry typically shows CD4+CD7- or CD4+CD26- phenotype. Management includes extracorporeal photopheresis, low-dose Methotrexate, and systemic therapies. Prognosis is poorer than patch/plaque stage mycosis fungoides.

Reference: BAD 2019 Guidelines for management of CTCL; EORTC-ISCL