A 60-year-old man presents with diffuse erythematous scaling plaques over the trunk progressing over years, now with circulating atypical cerebriform nuclei forming Pautrier microabscesses within the epidermis on skin biopsy. The malignant cells are CD4-positive T cells with loss of CD7. What is the diagnosis?
- A Sézary syndrome, a leukemic variant of mycosis fungoides ✓
- B Adult T-cell leukaemia/lymphoma
- C Peripheral T-cell lymphoma, NOS
- D Cutaneous anaplastic large cell lymphoma
Correct answer: A. Sézary syndrome, a leukemic variant of mycosis fungoides
Explanation
Pautrier microabscesses (collections of cerebriform T cells in the epidermis) are pathognomonic of mycosis fungoides, a cutaneous T-cell lymphoma of CD4-positive memory T cells. When it disseminates with widespread erythroderma and circulating Sézary cells, the entity is called Sézary syndrome. Adult T-cell leukaemia/lymphoma is HTLV-1 associated with hypercalcaemia and cloverleaf cells, and ALK-positive ALCL involves t(2;5).
Reference: Robbins and Cotran Pathologic Basis of Disease, 10th ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
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