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
Nikko AP, Rutherford CJ, Pandya AG. Successful Treatment of Sézary Syndrome With Lymphomatous Transformation to Large Cell Lymphoma With Fludarabine Phosphate. Arch Dermatol. 1996;132(8):978-979. doi:10.1001/archderm.1996.03890320128028