Welcome to the NORD Physician Guide to Cutaneous T-Cell Lymphoma (CTCL). The NORD Online Physician Guides are written for physicians by physicians with expertise on specific rare disorders. This guide was reviewed by Oleg E. Akilov, MD, PhD, Assistant Professor of Dermatology, Director, Cutaneous Lymphoma Program, University of Pittsburgh School of Medicine. (see acknowledgements for additional information).
NORD is a nonprofit organization representing all patients and families affected by rare diseases. The information NORD provides to medical professionals is intended to facilitate timely diagnosis and treatment for patients.
Cutaneous lymphoma is a general term for cancers of the lymphocytes that primarily involve the skin. They are a subtype of non-Hodgkin lymphoma. Cutaneous lymphoma is classified as either originated from B-lymphocyte (cutaneous B-cell lymphoma) or T-lymphocyte (cutaneous T-cell lymphoma). Approximately 75% of primary cutaneous lymphomas are T-cell derived.
This Physician Guide deals with cutaneous T-cell lymphoma (CTCL).
CTCL primarily affects the skin, but in more advanced disease can also involve the blood, lymph nodes, and various internal organs.
There are many types of CTCL. The two main types are mycosis fungoides (MF) and Sézary syndrome (SS) accounting for approximately 65% of all primary CTCL. The term mycosis fungoides originally referred to the mushroom-like nodules seen in the tumor stage.
The incidence of CTCL as a whole is currently estimated to be 6.4 per every 1 million people in the general population. According to Surveillance, Epidemiology, and End Results (SEER) registry data, males and African-Americans have the highest incidence rates.