Summary
Lymphapheresis was performed on a patient with Sézary syndrome. The Sézary cells were purified by removing E-rosette-forming and Fc receptor-bearing cells. Antiserum against these purified Sézary cells was raised in rabbits. This antiserum had cytotoxicity against Sézary cells as well as against normal peripheral blood lymphocytes. Absorption was carried out with chronic lymphocytic leukemia (CLL) and normal lymphocytes. The absorbed antiserum maintained cytotoxicity against Sézary cells but lost cytotoxicity against CLL and normal peripheral blood lymphocytes.
Indirect immunofluorescence assay showed that the antiserum reacted against purified Sézary cells and a high percentage (66%) of peripheral blood mononuclear cells from five patients with Sézary syndrome. It also reacted against 5.7% of normal lymphocytes, 8% of CLL cells, 5% of the lymphocytes from a patient who had undergone splenectomy, 2% of lymphocytes from a patient with multiple myeloma, 5% of lymphocytes from a hairy cell leukemia patient, and 1% of acute lymphocytic leukemia cells (T cell). The antiserum did not react against thymocytes but reacted against 34.6% of the bone marrow lymphocytes. This unique marker was designated as sigma (σ) antigen. It was suggested that Sézary syndrome may represent proliferation or malignant transformation of normally present σ antigen-positive lymphocytes.
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Khan, A., Burt, S.L.F., Hill, N.O. et al. A unique antigen (sigma) on sézary cells. Cancer Immunol Immunother 8, 225–230 (1980). https://doi.org/10.1007/BF00206040
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DOI: https://doi.org/10.1007/BF00206040