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.
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