Sézary Syndrome, T-Helper 2 Cytokines and Accessory Factor-1 (AF-1)

Abstract

Mycosis fungoides and the Sézary Syndrome are characterized by clonal accumulation of well differentiated T-helper memory cells in the skin and, in the case of the Sézary Syndrome, also in the blood and lymph nodes. Well known immunological abnormalities in patients with MF and SS include reduced delayed type hypersensitivity, decreased proliferation upon stimulation with mitogens of the peripheral blood mononuclear cells, elevated IgE or IgA serum levels and eosinophilia. These abnormalities can be explained by the predominance of T-helper 2 cytokines. Clonal T-lymphocytes purified from the peripheral blood of SS patients transcribe mainly IL-10 and IL-5. They can be CD7 positive or negative. These clonal T cells express the accessory factor-1 (AF-1) or Interferon γ receptor β-chain that is essential for Interferon γ signalling. These results imply perspectives for new therapeutical approaches, such as IL-12 or chimeric fusion proteins.

Key Words:

• Cutaneous T-cell lymphomas (CTCL)
• mycosis fungoides
• Sézary Syndrome
• IL-10
• IL-5
• T-helper 2
• accessory factor-1
• IFN-γ receptor β-chain