Sézary's syndrome: a cytogenetic, cytophotometric and autoradiographic study.
Cytophotometric, cytogenetic, and autoradiographic studies were performed in cells of three patients suffering from clinically diagnosed Sézary's syndrome with erythroderma and the presence of abnormal lymphoid cells in the peripheral blood, skin, bone marrow and lymph-nodes. Feulgen DNA cytophotometry of cells in the peripheral blood and skin lesions showed marked aneuploidy and tetraploidy. Multiple translocations were identified with a G-banding technique. The chromosomal abnormalities varied widely between the patients, but C and D group chromosomes were more frequently involved than others. All breakpoints of the translocations were localised in the centromeric region. Autoradiography of blood and skin samples revealed many labelled cells in the skin and a lower number in the blood, indicating cell proliferation in the skin. It is concluded that the pathological cells occurring in the Sézary syndrome are abnormal lymphoid cells with a tendency to proliferate in the dermis. The variability observed between and in the patients is in all probability due to a difference in the degree of dedifferentiation.
D000368:Aged; D001345:Autoradiography; D002869:Chromosome Aberrations / Q000473:pathology; D025063:Chromosome Disorders; D003873:Dermatitis, Exfoliative / Q000097:blood / Q000235:genetics / Q000473:pathology*; D005260:Female; D006801:Humans; D007645:Keratoderma, Palmoplantar / Q000097:blood / Q000235:genetics / Q000473:pathology; D008206:Lymphatic Diseases / Q000097:blood / Q000235:genetics / Q000473:pathology*; D008297:Male; D008854:Microscopy, Electron; D008875:Middle Aged; D010783:Photometry; D012878:Skin Neoplasms / Q000097:blood / Q000235:genetics / Q000473:pathology*; D013577:Syndrome