Case Studies

 Case 6.3 Sézary syndrome

A 66-year-old man presented with generalized itching and redness of his skin for 2 months, with severe keratosis on the soles of his feet and the palms of his hands. On examination, he had characteristic exfoliative dermatitis with bilateral axillary lymphadenopathy but no hepatosplenomegaly. Investigation showed that his haemoglobin was normal (139g/l) but he had a raised white-cell count (12.8 x 10⁹/l). A blood film showed an increase of small cleaved lymphocytes, 90% of which were T lymphocytes (see Table C6.2). Electron microscopy of buffy coat cells confirmed that the nuclei of these cells had multiple clefts, an appearance characteristic of cells known as Sézary cells. The patient had early-stage Sézary syndrome and was treated with psoralens and ultraviolet A therapy (PUVA) to the skin.