Dendritic cells in the dermal infiltrate of Sézary syndrome

The dermal infiltrates of four patients with the Sézary syndrome were studied by electron microscopy and the data were evaluated quantitatively. The nuclear contour index of lymphocytes was calculated, and many tumour cells had an index greater than 6.5. Dendritic cells were found in all cases. The dendritic cells contained smooth and rough endoplasmic reticulum, moderately well-developed Golgi apparatus, scanty lysosomes and many thin and intermediate filaments; their surface was scalloped with numerous vesicles. Birbeck granules were not found in the cytoplasm of dendritic cells. Dendritic cells comprised 24% of infiltrating cells and were interspersed with lymphocytes; 75% of the lymphocytes were in contact with dendritic cells; 35% of the lymphocytes in contact with dendritic cells had a nuclear contour index higher than 6.5 and 76% had a nuclear contour index higher than 5. The data strongly suggest a functional relationship between lymphocytes and dendritic cells in the dermal infiltrate of Sézary syndrome. They are discussed in relation to the hypothesis that the disease is a consequence of chronic immune stimulation.