Abstract: A 41-year-old male presented with diffuse erythema with severe pruritus for 1 year and multiple lymph node enlargement for 3 months. PET/CT image was consistent with lymphoma, pathology of lymph node showed non-Hodgkin T cell lymphoma, with irregular, twisted, and cerebriform nuclei. Skin pathology showed non-Hodgkin T cell lymphoma presenting with mycosis fungoides. Sézary syndrome was diagnosed according the above examinations and T cell gene rearrangement in peripheral blood. The effect of chemotherapy and other first-line drugs was not good, then the treatment regimen containing brentuximab vedotin was given, with good effect of erythema subsided, pruritus disappeared, lymph nodes decreased.

Key words: Sézary syndrome, cutaneous T-cell lymphoma, brentuximab vedotin