skip to main content

Sézary Syndrome — SCE Dermatology MCQ

Instant feedback + full explanation. One question, done properly.

HardSkin CancerSézary SyndromeSCE Dermatology

A 60-year-old man has CTCL. His dermatologist explains that Sézary syndrome is the leukaemic variant of CTCL. What defines Sézary syndrome?

Educational content. Not a substitute for clinical judgement or local policy.

Reveal the answer and explanation

Correct answer: A — The triad of erythroderma, generalised lymphadenopathy, and circulating neoplastic T cells (Sézary cells) in the peripheral blood — defined by specific blood involvement criteria

Sézary syndrome is defined by the triad of: (1) erythroderma (≥80% BSA); (2) generalised lymphadenopathy; (3) significant blood involvement with neoplastic Sézary cells — defined as: absolute Sézary cell count ≥1000/μL, OR CD4:CD8 ratio ≥10 (by flow cytometry), OR demonstration of T-cell clonality in blood by TCR gene rearrangement. Sézary cells are large atypical T lymphocytes with cerebriform (convoluted) nuclei. Sézary syndrome has a worse prognosis than erythrodermic MF (5-year survival approximately 25-40%). Treatment includes ECP (extracorporeal photopheresis — first-line), low-dose Methotrexate, HDAC inhibitors, Alemtuzumab, and mogamulizumab (anti-CCR4).

Reference: BAD 2019 CTCL; EORTC-ISCL