Systemic Hodgkin's lymphoma in a patient with Sezary syndrome

We report a case of a 71-year-old male with Sezary syndrome diagnosed in 19 96 who subsequently developed systemic Hodgkin's lymphoma. His only past tr eatment was bath psoralen plus ultraviolet A. He has since been treated wit h multiagent chemotherapy (ChlVPP/PABLOE) which induced a remission in his Hodgkin's disease. Eighteen months later he remains in remission from Hodgk in's disease but the Sezary syndrome remains active. He has also developed a squamous cell carcinoma on the upper lip. Sezary syndrome is a primary cu taneous T-cell lymphoma characterized by a malignant proliferation of CD4-p ositive cells in the skin and peripheral circulation. The CD4 count may be markedly elevated but this results from expansion of a neoplastic T-cell cl one and there is a relative lymphopenia of normal T cells leading to a degr ee of immunoparesis. Immunosuppression is known to be associated with an in creased rate of malignancies and this may account for the occurrence of Hod gkin's disease and squamous cell carcinoma in this patient with Sezary synd rome.