Merck Manual

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Cutaneous T-cell Lymphomas

(Mycosis Fungoides; Sézary Syndrome)

By

Peter Martin

, MD, Weill Cornell Medicine;


John P. Leonard

, MD, Weill Cornell Medicine

Reviewed/Revised Jun 2022 | Modified Sep 2022
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Topic Resources

The most common types of CTCL are

  • Mycosis fungoides

  • Sézary syndrome

Most people who develop CTCL are older than 50. It originates from mature T cells (T lymphocytes) and first affects the skin.

Mycosis fungoides starts so subtly and grows so slowly that it may not be noticed initially. It causes a long-lasting, itchy rash—sometimes a small area of thickened, itchy skin that later develops nodules and slowly spreads. In some people, it develops into a form of leukemia (Sézary syndrome). In other people, it progresses to the lymph nodes and internal organs. Even with a biopsy, doctors have trouble diagnosing this disease in its early stages. However, later in the course of the disease, a biopsy shows lymphoma cells in the skin.

Sézary syndrome also starts subtly and grows slowly. It causes the skin to become red all over the body with cracking of the palms and soles. Lymph node enlargement is usually mild. In addition to the rash, people may also have symptoms of fever, night sweats, and weight loss. As with mycosis fungoides, doctors have trouble diagnosing this disease in its early stage even with a skin biopsy. A blood smear (where a drop of blood is examined under a microscope) may show Sézary cells (malignant T cells with a characteristic appearance) and this can help make the diagnosis in addition to a skin biopsy.

Treatment of CTCL can be divided into

  • Therapy directed at the skin, such as light therapy (phototherapy) or topical drugs

  • Bodywide therapy, such as chemotherapy or targeted drugs, usually given intravenously

More Information

The following is an English-language resource that may be useful. Please note THE MANUAL is not responsible for the content of this resource.

NOTE: This is the Consumer Version. DOCTORS: VIEW PROFESSIONAL VERSION
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