A 68-year-old man presents with generalized erythroderma, intense pruritus, and generalized lymphadenopathy. Peripheral smear shows atypical lymphocytes with convoluted cerebriform nuclei. Skin biopsy demonstrates epidermotropism of atypical CD4-positive T cells forming collections within the epidermis. Which is the most likely diagnosis?
- A Sezary syndrome ✓
- B Adult T-cell leukemia/lymphoma
- C Angioimmunoblastic T-cell lymphoma
- D T-cell prolymphocytic leukemia
Correct answer: A. Sezary syndrome
Explanation
Erythroderma, circulating cerebriform (Sezary) cells, and Pautrier microabscesses, which are intraepidermal clusters of atypical CD4-positive T cells, define Sezary syndrome, the leukemic and erythrodermic variant of mycosis fungoides. Adult T-cell leukemia/lymphoma also shows highly pleomorphic circulating cells and hypercalcemia related to HTLV-1, and angioimmunoblastic lymphoma shows a polymorphous node with arborizing vasculature, neither of which fits this presentation.
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.