Primary cutaneous T-cell lymphomas.
Primary cutaneous T-cell lymphomas (CTCLs) encompass a clinically and biologically heterogeneous group of non-Hodgkin lymphomas (NHLs) defined by clonal proliferation of skin-homing malignant T lymphocytes and natural killer cells. They account for up to 75% to 80% of all cutaneous lymphomas. The current WHO-EORTC classification of cutaneous lymphomas with primary cutaneous manifestations lists 13 entities. The most common subtypes-mycosis fungoides, Sézary syndrome, primary cutaneous anaplastic large cell lymphoma, and lymphomatoid papulosis-which represent approximately 95% of CTCLs, will be discussed in the following review. Each entity has unique biological characteristics and clinical course. Topical and/or systemic therapies are employed based on the stage of the disease and the tempo of progression.
Rosen
Steven T
ST
Robert H. Lurie Comprehensive Cancer Center, Northwestern University, 710 N Fairbanks Ct, Olson Pavilion, Suite 8-250, Chicago IL 60611-2645, USA. s-rosen@northwestern.edu
Querfeld
Christiane
C
D002999:Clone Cells; D006801:Humans; D007694:Killer Cells, Natural / Q000473:pathology; D016410:Lymphoma, T-Cell, Cutaneous / Q000145:classification* / Q000473:pathology / Q000628:therapy; D013601:T-Lymphocytes / Q000473:pathology