This is a cutaneous T cell lymphoma characterised by erythroderma, leukaemia, generalised lymphadenopathy, and hepatosplenomegaly. Sezary syndrome is the leukaemic presentation of mycosis fungoides in which there are circulating malignant lymphoid cells in the peripheral blood (1).
It occurs most frequently in middle-aged males and histologically, is characterised by Sezary cells - CD4+ T lymphocytes with a highly convoluted and bizarre morphological appearance.
Patienst with Sézary syndrome have a median survival of less than 5 years (2).
- Lorincz AI. Cutaneous T-cell lymphoma (mycosis fungoides). Lancet 1996; 347: 871-876.
- NICE (February 2006).CSG Improving Outcomes for People with Skin Tumours including Melanoma: The Manual