Sézary syndrome (SS), a leukemic form of cutaneous T-cell lymphoma (CTCL), is highly resistant to contemporary therapeutic modalities. A more aggressive form of disease that is even less responsive to therapy and characterized by transformation to large cell lymphoma develops in a substantial percentage of patients with SS. We describe a patient with SS that underwent lymphomatous transformation to large cell lymphoma. Treatment with the nucleoside analogue fludarabine phosphate resulted in complete remission for 14 months.
Report of a Case.
A 72-year-old white woman sought treatment because of an 11-month history of a chronic, relapsing, intensely pruritic eruption that had progressed to a generalized erythroderma. She had been treated with topical and systemic corticosteroids, oral psoralen with UV-A, etretinate, and hydroxyurea without substantial improvement. A physical examination revealed diffuse erythema and multiple, widespread, thin, scaly plaques. Erythema and keratoderma of her palms and soles were present. Laboratory data revealed