A 63-year-old man has diffuse erythroderma with intense pruritus, palmoplantar keratoderma, and generalized lymphadenopathy. Peripheral smear reveals atypical lymphocytes with convoluted, cerebriform nuclei. Flow cytometry shows CD3+ CD4+ cells with loss of CD7 constituting 40% of lymphocytes. Which diagnosis fits best?
- A Sézary syndrome ✓
- B Adult T-cell leukaemia/lymphoma
- C Angioimmunoblastic T-cell lymphoma
- D Large granular lymphocyte leukaemia
Explanation
Sézary syndrome is the leukaemic variant of mycosis fungoides, defined by erythroderma, lymphadenopathy, and circulating cerebriform (Sézary) cells; loss of pan-T-cell markers such as CD7 on CD4+ cells supports clonality. Adult T-cell leukaemia/lymphoma is linked to HTLV-1 and typically shows flower-like multilobated nuclei and hypercalcaemia. Angioimmunoblastic T-cell lymphoma features systemic symptoms and polyclonal hypergammaglobulinaemia, while large granular lymphocyte leukaemia expresses CD3 with CD8 or NK markers, not a CD4-dominant erythrodermic phenotype.
Reference: Robbins and Cotran Pathologic Basis of Disease, 10th ed.
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Written and medically reviewed by the StethoPrep medical team.