Systemic Hodgkin’s lymphoma in a patient with Sézary syndrome

Abstract

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.

Practical evaluation and management of cutaneous lymphoma

“…SS may also be associated with lymphopenia that may correlate with an increased incidence of secondary malignancy including squamous cell carcinoma of the skin or oral mucosa, or HD. 64,65 Large T-cell lymphoma, CD30 + /CD30 -Termed anaplastic large-cell lymphoma in the REAL classification, EORTC dropped the "anaplastic" from these histologically distinctive lymphomas because not every such tumor histologically exhibits anaplastic cells. 66 Other designations include largecell anaplastic lymphoma and regressing atypical histiocytosis.…”