Cutaneous T-cell Lymphoma (CTCL) Overview

Cutaneous T-cell lymphoma is a type of non-Hodgkin lymphoma that primarily affects the skin. Also known as cutaneous lymphoma and lymphoma of the skin, the cancer originates in certain white blood cells involved in the body’s immune response (T-lymphocytes), which are found in the skin and lymphatic tissues throughout the body. Cutaneous T-cell lymphoma is typically slow-growing and can develop over many years.

Overall, cutaneous T-cell lymphoma is relatively rare. According to the Cutaneous Lymphoma Foundation, approximately 3,000 new cases are reported in the United States each year.

There are two main types of cutaneous lymphoma: mycosis fungoides, which is more common, and Sezary syndrome, which some experts consider to be an advanced stage of mycosis fungoides.

Sezary syndrome vs. mycosis fungoides

The primary difference between the two main types of cutaneous lymphoma is that with Sezary syndrome, cancerous T-cells are found in the blood as well as the skin.

What causes cutaneous T-cell lymphoma?

Cutaneous T-cell lymphoma begins with harmful changes to T-lymphocytes that cause them to grow uncontrollably. The triggers of the cellular DNA mutations that lead to the development of lymphoma of the skin are not well understood. Scientists believe several factors may play a role in its development, but a direct causal link has not yet been established.

What are the risk factors for cutaneous T-cell lymphoma?

Known risk factors for lymphoma of the skin include:

• Advanced age – Cutaneous T-cell lymphoma primarily affects men in their 50s and 60s, although it can also occur in children, young adults and women of all ages.
• Compromised immune system – Individuals who take medication to prevent organ rejection after a transplant or have an infection such as acquired immunodeficiency syndrome (AIDS) may be at heightened risk for developing lymphoma of the skin.
• Certain viral infections – Human T-cell lymphotropic virus type 1 (HTLV-1) is associated with an elevated risk of cutaneous lymphoma.
• Genetics – A family history of lymphoma or other blood cancers may increase the risk, suggesting a potential genetic predisposition.
• Chronic inflammation – Conditions that cause long-term inflammation of the skin, such as eczema and other inflammatory skin disorders, may increase the risk of cutaneous lymphoma.
• Exposure to chemicals or radiation – Prolonged exposure to certain harmful substances, such as pesticides and industrial chemicals, and prior radiation therapy for cancer treatment may increase the risk.

What are the symptoms of cutaneous T-cell lymphoma?

The first sign of mycosis fungoides is typically a skin rash. Without treatment, the rash may become itchy or develop ulcers. Other symptoms characteristic of both mycosis fungoides and Sezary syndrome include:

• Round skin patches that may be raised, scaly or itchy
• Tumors on or just beneath the surface of the skin
• Skin patches that are lighter in color than the surrounding skin
• Enlarged lymph nodes
• Extremely itchy skin with redness covering most of the body (erythroderma)

Additional Sezary syndrome symptoms can include:

• Thick, cracked skin on the hands or feet (keratoderma)
• Frequent staph infections
• Changes in hair, nails or eyelids
• Increased susceptibility to feeling cold

The symptoms of early-stage cutaneous T-cell lymphoma can be similar to those of eczema, psoriasis and other benign skin conditions. An accurate diagnosis typically requires blood testing and one or more skin biopsies.

How is cutaneous T-cell lymphoma diagnosed?

The diagnostic process for lymphoma of the skin typically involves several steps, which may include:

• Physical examination – A physician will examine the skin for lesions, plaques and other changes, which can provide initial clues about cutaneous lymphoma.
• Skin biopsy – A small sample of suspicious tissue will be removed and microscopically examined by a pathologist, who can identify abnormal T-cells.
• Blood work – A complete blood count (CBC) and flow cytometry can reveal abnormalities in the bloodstream that may be associated with cutaneous lymphoma.
• Imaging – A physician may order computed tomography (CT), magnetic resonance imaging (MRI) or positron emission tomography (PET) scans to check for cancer spread to lymph nodes or internal organs, especially in advanced stages.
• Molecular and genetic testing – T-cell receptor gene rearrangement studies can help a physician confirm the presence of clonal T-cell populations specific to lymphoma of the skin.

Cutaneous T-cell lymphoma can be challenging to distinguish from benign skin conditions. Therefore, an accurate diagnosis often requires careful analysis of biopsy samples and lab results.

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How is cutaneous T-cell lymphoma treated?

Treatment for lymphoma of the skin can vary from patient to patient but may include:

• Skin-directed therapies:
  • Topicals – Creams, lotions, ointments or gels can be applied to the affected skin.
  • Phototherapy – Ultraviolet light can be used to destroy lymphoma cells in the skin.
  • Radiation therapy – High-energy beams of radiation can be used to target and destroy cancerous cells.
• Immunotherapy – Genetically modified cells or man-made antibodies can be introduced into the body to help the immune system identify and attack cancerous cells or inhibit their growth.
• Photopheresis – Lymphoma cells are collected, exposed to ultraviolet (UV) light to modify their abnormal activity and then reinfused into the patient.
• Chemotherapy – Powerful cancer-fighting medications can be delivered intravenously or orally.
• Bone marrow transplantation – Abnormal cells in the blood and bone marrow can be replaced with healthy new cells.
• Clinical trials – Scientists continue to perform research studies to explore and refine treatments for cutaneous lymphoma.

Benefit from world-class care at Moffitt Cancer Center

Moffitt is a high-volume cancer center that takes a multispecialty approach to diagnosing and treating cutaneous T-cell lymphoma. Our diverse team comprises experts from all specialties within our Malignant Hematology and Cutaneous Oncology Programs, including dermatologists, medical oncologists, radiation oncologists, dermatopathologists, surgical oncologists, supportive care specialists, researchers and many other professionals. Working together, we develop an individualized treatment plan for each patient tailored to their unique needs and preferences. As a National Cancer Institute-designated Comprehensive Cancer Center, Moffitt is widely recognized for its robust clinical trials program, which allows our patients to access many groundbreaking therapies before those options are made available elsewhere.

If you would like to learn more about cutaneous T-cell lymphoma, you can request an appointment with a specialist at Moffitt by calling 1-888-663-3488 or submitting a new patient registration form online. We do not require referrals.

References

Mycosis Fungoides and Sézary Syndrome - PMC
Mycosis Fungoides (Including Sézary Syndrome) Treatment (PDQ®)–Patient Version - NCI
Mycosis Fungoides | Cutaneous Lymphoma Foundation