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An aggressive form of cutaneous T-cell lymphoma in which the skin is diffusely involved and there are abnormal cells in the peripheral blood.
Of estimated 3,000 cases of mycosis fungoides each year, approximately 155 have Sezary syndrome.
Usually occur in people over 50 years, but can occur at any age.
Slighly more common in men.
No known risk factors.
Distinguished from mycosis fungoides by the presence of malignant lymphocytes in the blood and is characterized by extensive thin red, itchy rashes covering over 80% of the body.
May be associated with thicker red plaques and tumors, and may be accompanied by changes in nails, eyelids or enlaged lymph nodes.
Makes up 3% of cutaneous T-cell lymphoma cases.
Disproportionally poor survival among black population.
Overall 5-yer survival for whites about 44% and for Blacks <16% (Jang S et al).