[The Sézary syndrome--an erythrodermic T-cell-lymphoma]

Report on a 75-year-old patient with Sézary syndrome which is characterized by the following leading symptoms: Erythrodermia, leukocytosis, circulating atypical lymphocytes with cerebriformous nuclei (Sézary cells) and swollen lymph nodes. Light microscopic and electron microscopic findings refer to a transitory situation of the Sézary cells from the reactive character to the (pre)neoplastic one. The disease is classified by T-(helper)cells as a low grade non-Hodgkin-lymphoma in parallel to mycosis fungoides and in general it finishes lethally after a course of 5 to 8 years. Apart from local steroid ointments and UV-irradiations the internal application of steroids, in the late stage if necessary in combination with cytostatics is recommended for therapy.