Systemic Hodgkin's lymphoma in a patient with Sézary syndrome.
We report a case of a 71-year-old male with Sézary syndrome diagnosed in 1996 who subsequently developed systemic Hodgkin's lymphoma. His only past treatment was bath psoralen plus ultraviolet A. He has since been treated with multiagent chemotherapy (ChlVPP/PABLOE) which induced a remission in his Hodgkin's disease. Eighteen months later he remains in remission from Hodgkin's disease but the Sézary syndrome remains active. He has also developed a squamous cell carcinoma on the upper lip. Sézary syndrome is a primary cutaneous T-cell lymphoma characterized by a malignant proliferation of CD4-positive cells in the skin and peripheral circulation. The CD4 count may be markedly elevated but this results from expansion of a neoplastic T-cell clone and there is a relative lymphopenia of normal T cells leading to a degree of immunoparesis. Immunosuppression is known to be associated with an increased rate of malignancies and this may account for the occurrence of Hodgkin's disease and squamous cell carcinoma in this patient with Sézary syndrome.
Scarisbrick
J J
JJ
Skin Tumour Unit, St John's Institute of Dermatology, St Thomas' Hospital, Lambeth Palace Rd, Westminster, London SE1 7EH, UK.
Child
F
F
Spittle
M
M
Calonje
E
E
Russell-Jones
R
R
D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002294:Carcinoma, Squamous Cell / Q000188:drug therapy / Q000276:immunology / Q000473:pathology; D006689:Hodgkin Disease / Q000188:drug therapy / Q000276:immunology / Q000473:pathology; D006801:Humans; D007108:Immune Tolerance / Q000276:immunology; D008048:Lip Neoplasms / Q000188:drug therapy / Q000276:immunology / Q000473:pathology; D008297:Male; D009378:Neoplasms, Multiple Primary / Q000188:drug therapy / Q000276:immunology / Q000473:pathology; D012751:Sezary Syndrome / Q000188:drug therapy / Q000276:immunology / Q000473:pathology; D012878:Skin Neoplasms / Q000188:drug therapy / Q000276:immunology / Q000473:pathology; D016896:Treatment Outcome