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Hematology 2006
© 2006 The American Society of Hematology

Primary Cutaneous T-Cell Lymphomas

Steven T. Rosen and Christiane Querfeld

Correspondence: Steven Rosen, MD, Robert H. Lurie Comprehensive Cancer Center, Northwestern University, 710 N, Fairbanks Ct, Olson Pavilion, Suite 8-250, Chicago IL 60611-2645; Phone 2312-908-5250; Fax 312-908-1372; Email s-rosen{at}northwestern.edu

Abstract

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.


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Copyright © 2006 by the American Society of Hematology.