hematology and oncology
Cutaneous T cell Lymphomas (CTCL)
Introduction
Mycosis fungoides and Sézary syndrome are uncommon chronic T-cell non-Hodgkin lymphomas primarily affecting the skin and occasionally the lymph nodes. (See also Overview of Lymphoma and Mycosis fungoides and Sézary syndrome are the 2 main types of cutaneous T-cell lymphomas. They comprise less than 5% of all lymphoma cases. Cutaneous T-cell lymphomas are insidious in onset. Patients may initially present with a chronic, pruritic rash that is difficult to diagnose even with biopsies. This prodrome may exist for several years until the diagnosis of cutaneous T-cell lymphoma is finally made. The lesions of mycosis fungoides are characterized as patches, plaques, or tumor nodules; the nodules often ulcerate and become infected. In Sézary syndrome, the skin is typically diffusely red with cracking of the palms and soles. Lymphadenopathy is usually mild to moderate. Symptoms are related mostly to the skin, with fevers, night sweats, and unintentional weight loss coming later in the disease course. This photo shows red patches on the back of a patient with mycosis fungoides. DR P. MARAZZI/SCIENCE PHOTO LIBRARY Image courtesy of Karen McKoy, MD. Image courtesy of Karen McKoy, MD. Mycosis fungoides (cutaneous T-cell lymphoma) can be difficult to distinguish from nonmalignant chronic dermatoses. Accurate diagnosis can be made only by a detailed clinical history (no progression of nonmalignant lesions over time) and biopsy with microscopic evaluation. (Courtesy of Libby Edwards, MD, Charlotte, NC.) By permission of the publisher. From Banks P, et al. InAtlas of Clinical Hematology. Edited by JO Armitage. Philadelphia, Current Medicine, 2004. Available at www.images.md. Ulcerations can sometimes develop in patients with mycosis fungoides. DR P. MARAZZI/SCIENCE PHOTO LIBRARY 