title: Cutaneous T-cell lymphomas: the Sézary syndrome, mycosis fungoides, and related disorders.
abstract: Substantial evidence has accumulated to indicate not only that mycosis fungoides and the  Sézary syndrome are closely related malignancies, but to suggest that they are part of a larger spectrum of cutaneous lymphomas. The neoplastic cells of these disorders have membrane features of thymus-derived (T) lymphocytes, a characteristic tissue distribution (skin infiltration, marrow sparing, localization in T-cell regions of lymphoid tissue), and distinctive morphology. For these reasons, we suggest that these lymphoproliferative disorders be grouped together as "cutaneous T-cell lymphomas". The anergy noted in patients of this group with leukemia probably is related to both decreased percentages of normal T cells and presence in the serum of macrophage migration inhibitory activity. Leukapheresis has been particularly effective in the management of selected patients. The homogeneous T-cell populations in the patients with leukemia also provide important opportunities to study many aspects of lymphocyte physiology that are of broad biologic significance.
mesh_terms: D002462:Cell Membrane / Q000473:pathology; D003873:Dermatitis, Exfoliative / Q000097:blood / Q000276:immunology / Q000473:pathology; D005260:Female; D006801:Humans; D007645:Keratoderma, Palmoplantar / Q000097:blood / Q000276:immunology / Q000473:pathology; D008206:Lymphatic Diseases / Q000097:blood / Q000276:immunology / Q000473:pathology; D008214:Lymphocytes / Q000473:pathology; D008223:Lymphoma / Q000276:immunology / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009182:Mycosis Fungoides / Q000097:blood / Q000276:immunology / Q000473:pathology; D012867:Skin / Q000473:pathology*; D013577:Syndrome; D013601:T-Lymphocytes / Q000473:pathology