A 62-year-old man presents with progressive generalized erythroderma, pruritus, palmoplantar keratoderma, and diffuse lymphadenopathy. Peripheral blood shows atypical lymphocytes with deeply convoluted, cerebriform nuclei comprising 30% of leukocytes. Flow cytometry demonstrates CD4+ T cells with loss of CD7 expression. Which of the following is the most likely diagnosis?
- A Angioimmunoblastic T-cell lymphoma
- B Adult T-cell leukemia/lymphoma
- C Sezary syndrome ✓
- D Mycosis fungoides, patch stage
Correct answer: C. Sezary syndrome
Explanation
Sezary syndrome is the leukemic and erythrodermic variant of mycosis fungoides, defined by erythroderma, generalized lymphadenopathy, and circulating Sezary cells with characteristic cerebriform nuclei; the neoplastic cells are mature CD4+ T cells that typically lose CD7. Adult T-cell leukemia/lymphoma is linked to HTLV-1 and shows polylobated flower cells rather than cerebriform nuclei. Patch-stage mycosis fungoides lacks erythroderma and blood involvement.
Reference: Robbins and Cotran Pathologic Basis of Disease, 10th ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
Written and medically reviewed by the StethoPrep medical team.