A 70-year-old man presents with erythroderma of 4 weeks duration. He has palpable cervical, axillary, and inguinal lymphadenopathy. Skin biopsy shows band-like infiltrate of atypical lymphocytes with epidermotropism and Pautrier microabscesses. Peripheral blood flow cytometry shows CD4+ T-cells with cerebriform nuclei comprising 25% of lymphocytes. What is the diagnosis?
- A Mycosis fungoides in tumor stage
- B Primary cutaneous anaplastic large cell lymphoma
- C Sézary syndrome ✓
- D Adult T-cell leukemia-lymphoma
Correct answer: C. Sézary syndrome
Explanation
Sézary syndrome is defined by the triad of erythroderma, lymphadenopathy, and clonal Sézary cells (CD4+ T-cells with cerebriform nuclei) in blood, skin, and lymph nodes. The presence of >5% or >1000/μL Sézary cells in peripheral blood confirms the diagnosis. The skin biopsy showing Pautrier microabscesses and epidermotropism is characteristic. Mycosis fungoides lacks the leukemic blood component. Option C captures all diagnostic criteria.
Reference: WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues, 5th ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
Written and medically reviewed by the StethoPrep medical team.