Abstract
We report a 66-year-old man with Sézary syndrome (SzS) treated with natural interferon-gamma (nIFN-γ). He first visited our hospital on August 30, 1999, complaining of pruritic erythema on his trunk. Although the topical therapy with steroids was continued, he presented with gradually increasing generalized erythroderma. On physical examination, enlarged lymph nodes were palpable in his neck, axilla, and groin. Biopsy specimens of the left groin lymph nodes showed dermatopathic lymphadenitis, but the skin specimen revealed dense upper dermal band-like infiltration composed of atypical lymphocytes. The white blood cell count was 17,700/μl with 21.5% atypical lymphoid cells with prominent nuclear convolutions and infoldings typical of Sézary cells. The DNA gene rearrangement study of the peripheral blood showed identical clonal rearrangements of the TCR βchain gene. We diagnosed him as SzS. The combination therapy of prednisolone and nIFN-γ was very effective, and the patient responded clinically with complete clearance of his skin and disappearance of the lymphadenopathy. There were no severe side effects during treatment. The patient currently remains in complete clinical remission about one year since his diagnosis.
