Fulminant cutaneous T-cell lymphoma and Sézary syndrome.
The term cutaneous T-cell lymphoma (CTCL) has recently been coined to describe a group of rare lymphoproliferative T-cell disorders classically beginning in the skin and pursuing a chronic, progressive, and indolent course eventually followed by visceral involvement. This term encompasses the spectrum of the traditional mycosis fungoides and Sézary syndrome. A unique case of CTCL that developed a fulminant course leading to death within a few months following the initial symptoms is presented. The clinical course bears a striking similarity to a group of acute T-cell malignancies, while the cytologic-pathologic findings favor the diagnosis of Sézary syndrome. Regardless of the exact classifications, this entity appears to be a highly malignant T-cell disorder, resistant to chemotherapy.
D000328:Adult; D005260:Female; D006801:Humans; D008223:Lymphoma / Q000150:complications* / Q000276:immunology / Q000473:pathology; D012397:Rosette Formation; D012751:Sezary Syndrome / Q000150:complications* / Q000276:immunology / Q000473:pathology; D012867:Skin / Q000473:pathology; D012878:Skin Neoplasms / Q000150:complications* / Q000276:immunology / Q000473:pathology; D013601:T-Lymphocytes / Q000276:immunology / Q000648:ultrastructure