Cutaneous T-cell lymphoma (CTCL) is an indolent (low grade)
T-cell lymphoma that usually starts in or on the skin. Mycosis Fungoides is the most common type and classic presentation of CTCL and usually progresses slowly over many years. In rare cases, affected individuals may develop Sezary syndrome, a leukemic variant of mycosis fungoides.
CTCL should not be confused with peripheral T-cell lymphomas (PTL) or adult T-cell lymphoma/leukemia (ATLL) which are aggressive lymphomas which may also present themselves in the skin but require different types of treatment.
Diagnosis and Staging
Once the doctors suspect a lymphoma diagnosis they will perform tests to confirm the diagnosis.
This will include one or more biopsies (surgical removal of suspect tissue) which will be examined by an experienced pathologist to determine the type of cancer and how far it has spread. The determination of the spread of the disease is called staging and for CTCL it is different than for other types of Non-Hodgkin's lymphoma. The TNM classification is used. The cancer is characterized by the spread of the tumors on the skin (from T1 to T4), the lymph node involvement (N0 to N3) and whether thee are distant metastasis (spread to visceral organs) M1 or not M0.
Treatment
Treatment depends on the stage of the disease. Early stage (limited) disease may be treated with chemotherapy applied to the skin and ultraviolet A light exposure (PUVA). Total skin electron beam radiation (TSEB) may be used for disease that has spread. Treatment with multiple agent chemotherapy may also be used and there are clinical trials testing combinations of treatment.
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