Sézary disease

Sézary disease is sometimes considered a late stage of mycosis fungoides with lymphadenopathy.

[3][4] Sézary disease and mycosis fungoides are cutaneous T-cell lymphomas having a primary manifestation in the skin.

[5] The disease's origin is a peripheral CD4+ T-lymphocyte,[3] although rarer CD8+/CD4- cases have been observed.

[19] The FDA has approved denileukin diftitox-cxdl (Lymphir) for the treatment of patients with relapsed/refractory cutaneous T-cell lymphoma (CTCL) after at least 1 prior systemic therapy.

LYMPHIR (denileukin diftitox-cxcl) Injection 300 mcg is indicated for the treatment of adult patients with relapsed or refractory Stage I-III cutaneous T-cell lymphoma (CTCL) after at least one prior systemic therapy.

Sézary cell: pleomorphic abnormal T cell with the characteristic cerebriform nuclei (Peripheral blood - MGG stain)
Sézary syndrome in a 61-year-old man presenting in 1972 with unrelenting itchiness of six months' duration. On the right is his peripheral blood film stained with Periodic Acid-Schiff (PAS) showing a neoplastic T cell (Sézary cell).