Journal of Clinical Oncology, Vol 18, Issue 15
(August), 2000: 2908-2925
© 2000 American Society for Clinical Oncology
Primary Cutaneous T-Cell Lymphoma: Review and Current Concepts
By Richard S. Siegel,
Tomi Pandolfino,
Joan Guitart,
Steven Rosen,
Timothy M. Kuzel
From the Departments of Hematology/Oncology and Dermatology, Robert H. Lurie Comprehensive Cancer Center, Northwestern University Medical School, Chicago, IL.
Address reprint requests to Richard Siegel, MD, Department of Hematology and Oncology, Robert H. Lurie Comprehensive Cancer Center, 676 N St Clair, Suite 850, Chicago, IL 60611; email r_siegel{at}northwestern.edu © 2000 by American Society of Clinical Oncology. 0732-183X/00/1815-2908
ABSTRACT
PURPOSE: Primary cutaneous T-cell lymphomas (CTCLs) encompass a wide variety of lymphomas that are characterized by the localization of the malignant lymphocytes to the skin at presentation. Advances in molecular biologic techniques, including immunophenotyping and gene rearrangement studies to determine clonality, have led to more frequent diagnosis of CTCL as well as more consistent subclassification of these entities. However, there continues to be confusion in the classification, prognosis, and management of patients with CTCL. The purpose of this review is to present a summary of the diagnosis, prognosis, and treatment of CTCL, with specific emphasis on mycosis fungoides (MF) and Sézary syndrome (SS). We also present a detailed discussion of the entities that make up the differential diagnosis of CTCL.
DESIGN: We reviewed the medical literature on CTCL and other diseases that make up the differential diagnosis of CTCL.
Results and
CONCLUSION: MF and SS are the most common forms of CTCL. The etiology of this disease is still unknown. Patients may go for months to years with skin abnormalities before being diagnosed. MF/SS is an indolent disease and patients with T1 disease have a normal life expectancy. Patients who undergo transformation to large-cell lymphoma (8% to 23% of patients) have a poor prognosis, with mean survival ranging from 2 to 19 months. Treatment for MF/SS continues to be palliative. There are many new therapies that are currently being investigated in clinical trials, and the DAB389IL-2 fusion protein was recently approved for the treatment of refractory MF/SS.
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