Cutaneous T-Cell Lymphoma, Mycosis Fungoides, and Sezary Syndrome

sezary cell
Sezary cell in blood sample
Image by Herbert L. Fred, MD

Cutaneous T-Cell Lymphoma (CTCL) is a rare, low grade (indolent) type of Non-Hodgkin’s Lymphoma. It accounts for about one out of every twenty Non-Hodgkin’s diagnoses. This cancer is slow growing and mainly affects white blood cells called T-cells in the skin. While there are several types of CTCL, Mycosis Fungoides is the most common diagnosis.

Mycosis Fungoides is a classic presentation of CTCL. Symptoms include tumors that look like rashes and itching. Of course, these symptoms can be confused with eczema or another simple skin disorder. Proper tests are required to accurately diagnose and stage the disease.

Sézary Syndrome is a more serious type of CTCL affecting large areas of the skin. Lymph node involvement and the migration of cancerous T-cells into the blood are also markers of this advanced stage of CTCL.

Diagnosis and Staging

Cutaneous T-Cell Lymphomas are diagnosed using biopsies. During a biopsy, a doctor will remove a small portion of the diseased tissue for testing in the laboratory. For most CTCLs, this procedure can be done with local anesthetics and is relatively simple.

In the laboratory, a pathologist will examine the tissue and stage the disease according to TNM classification. Pathologists measure the spread of the tumors on the skin (T1 to T4), the involvement of the lymph nodes (N0 to N3), and the spread of affected T-cells to internal organs (M0 or M1). Using these classifications, the pathologist will stage the disease as follows:

Patch stage
This is the earliest stage of the disease, when it can be confused with eczema. Small, red, rash-like patches may appear on the skin, but the disease has not spread to the lymph nodes or internally.
Plaque Stage
Larger areas of the skin have become affected. The red patches in this stage are called plaques and most commonly occur in skin folds. No lymph involvement or internal spread has occurred yet in this stage.
Tumor Stage
The large, red patches on the patient’s skin may ulcerate or become painful in this stage. Affected T-cells may move into the lymph system. However, spread to the internal organs in this stage is very rare.

Treatment

There are many different treatments for CTCL. The choice of treatment usually depends on the stage of the disease.

Topical Treatments
Topical corticosteroid creams and ointments are frequently prescribed, especially in early stages of CTCL. Bexarotene gel is also a common treatment.
PUVA Treatment
Also known as photochemotherapy, PUVA treatment is successful in treating CTCL where large areas of the skin are affected. The patient takes a drug called psoralen (P), which makes the skin sensitive to Ultraviolet A light rays. The UVA then attacks cancerous cells.
UVB Therapy
This therapy is similar to PUVA treatment, except the patient does not need to take any sensitizing drugs. Skin is exposed to Ultraviolet B rays instead of Ultraviolet A, but the effect is similar.
Bexarotene capsules
Bexarotene in capsule form can slow or stall the growth of large CTCL tumors. The patient ingests the pill with food daily.
Photopheresis
Pheresis treatments involve removing blood from a patient, cycling it through a machine to clean or treat it, and then returning it to the patient. For Sézary Syndrome patients, the blood is exposed to UV light before it is returned to the body. This helps remove affected T-cells from the blood.
Chemotherapy
There are chemotherapy agents available to patients suffering from CTCL. Oftentimes they are applied in a lotion or cream directly to the skin (often referred to as "topical chemotherapy") to target affected areas. This will help slow or stop the growth of tumors. In rare cases of advanced CTCL, chemotherapy may also be injected into the patient’s blood stream.
Radiation
Radiation uses high energy x-rays or gamma rays to kill cancer cells. For CTCL in its early stages, radiation can be used to remove small tumor areas. If the tumors have spread to the lymph system, total skin electron beam treatment (a specialized form of radiation) may be used in combination with PUVA therapy. This treatment targets large areas of tumors as long as the cancer hasn’t spread internally.
Clinical Trials
There are many clinical trials for CTCL treatment. Some use drugs called inferons or retinoids to boost the effectiveness of PUVA treatments. Others use new chemotherapy agents to target large skin tumors. Talk to your doctor about clinical trials if you are interested in participating in one. Or, follow this link to learn more and find a clinical study opportunity near you.

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