Cutaneous T-Cell Lymphoma

Cutaneous T cell lymphoma (CTCL) is a rare type of cancer that affects the skin, blood and occasionally, lymph nodes and internal organs.  It occurs when a type of cell called a T lymphocyte grows out of control. Patients with mild cases may have only a rash. In more severe cases, tumors can form.

The cause of CTCL is not known, and only 5 to 10 people per million people are affected. It is more common in men than women and after age 50.

Progression of CTCL

The rate and extent of disease progression in patients with CTCL varies. The disease can progress slowly, rapidly or not all. Most patients have only skin symptoms with no serious complications. In about 10 percent of patients, progression spreads cancerous cells to the lymph nodes or internal organs. Most patients live normal lives throughout treatment and some experience long periods of remission.

While there is no cure for CTCL, people diagnosed and treated early have a normal life expectancy, so it’s important to see your dermatologist if you have symptoms.

Types of CTCL

Though there are many types of CTCL, the most common are mycosis fungoides and Sezary syndrome.

  • Mycosis Fungoides
    The most common type of CCLT, mycosis fungoides generally progresses slowly, often remaining confined to the skin. Symptoms appear anywhere on the skin in three phases: patch, plaque and tumor. Patients may have one or all three phases. Patches are usually flat, red and scaly and may be mistaken for eczema or dermatitis. Plaques are thicker, raised lesions. Tumors are larger lesions that can ulcerate continue to grow becoming mushroom-shaped.
  • Sézary Syndrome
    With Sézary Syndrome, most or all of the skin is covered in a red itchy rash, tumor cells are found in the blood and lymph nodes are enlarged. Patients may also have intense skin itching, flaking or burning; hair loss; thickening palms, fingernails and soles; drooping eyelids, loss of eyelashes; and difficulty closing the eyes.

Diagnosis

Along with reviewing a patient’s medical history and performing a physical examination, dermatologist diagnose CTCL using blood tests and skin biopsies. Because CTCL can be difficult to diagnose, a number of biopsies may be needed.

Treatment

Treatment for CTCL is designed to control symptoms, such as itching, burning, patches and skin tumors. With Sézary Syndrome, treatment goals are reducing skin redness and the number of abnormal cells in the blood.

Dermatologists recommend treatment based on the type of CTCL and severity of the disease as well as the patient’s health, age and lifestyle. Current treatment options include: topical medications applied to the skin, oral medication, systemic chemotherapy, light therapies, interferon injections and radiation.

Topical Medications

  • Cortisone
    Cortisone (corticosteroid) preparations temporarily control skin inflammation in many CTCL patients. Because of potential side effects, strong cortisone preparations should be used exactly as directed by your dermatologist and should not be stopped abruptly. CTCL may become resistant to cortisone with time.
  • Nitrogen Mustard
    Nitrogen mustard (ointment or liquid) is a type of topical chemotherapy that may clear skin temporarily and control CTCL. Nitrogen mustard is applied once daily as directed by the dermatologist. Skin irritation caused by an allergic reaction is a possible side effect.
  • Retinoids (Gel)
    Bexarotene gel, a retinoid derivative of Vitamin A, is an FDA-approved treatment for early-stage CTCL that acts by interfering with the growth of tumor cells. Side effects may include skin rash, redness and itching.

Oral Medications

  • Corticosteroids (oral)
    A powerful anti-inflammatory corticosteroid (usually prednisone) may be prescribed alone or in combination with other treatments to control CTCL. Due to potential of serious side effects, oral corticosteroids are usually reserved for severe cases of CTCL and patients should be monitored closely by a dermatologist.
  • Retinoids (capsule)
    The oral form of bexarotene is FDA-approved for advance-stage CTCL or patients who have not responded well to other therapies. The capsules, taken daily, act by causing cell death in cancerous T-Cells. The medication is usually well-tolerated, but regular blood tests are necessary to monitor for potential side effects.
  • Methotrexate
    Methotrexate is an oral anticancer drug used to control CTCL. Liver function should be monitored in patients taking methotrexate. Other possible side effects include upset stomach, nausea, mouth ulcers and dizziness.

Systemic Chemotherapy

Chemotherapy medications given intravenously travel through the bloodstream to kill cancer cells throughout the body. Many different types of drugs are used and side effects depend on the type of drug.

Fusion Protein is a type of targeted systemic therapy approved for recurring CTCL that uses interleukin-2 (IL-2) to attach to IL-2 receptors on malignant T-Cells and deliver a toxin that kills the cancer cells.

Light Therapies

  • Ultraviolet Light B (UVB) or Narrow-Band UVB Light
    Dermatologists use light boxes that expose the skin to ultraviolet rays to slow the rapid growth of skin cells safely and effectively.
  • PUVA
    PUVA combines a medication known as psoralen, which makes the skin very sensitive to light, and treatment with a specific type of ultraviolet light called UVA. Treatment is usually required three times a week and may take several months. Because psoralen also increases light sensitivity in the eyes, patients must wear UVA blocking eyeglasses on the day of treatment.
  • Extracorporeal Photopheresis (ECP)
    With ECP treatment, blood is taken from a vein circulated through a machine where it sensitized to light with psoralen and exposed to ultraviolet light then returned to the body, a process that selectively destroys cancer cells in the blood. Treatment usually requires visiting medical center for two days once a month.

All light therapies have potential side effects including a sunburn-like skin condition, premature aging, freckling and skin cancer.

Radiation

Spot radiation may be used to help kill cancer cells in targeted areas of the skin, or the entire skin surface can be treated with radiation. Side effects include skin inflammation, hair and nail loss, and fatigue.

Interferon

Interferon is a medication given by injection three to five times per week to control tumor growth. Injections can be given by patient or someone else at home or by a dermatologist. Side effects, including flu-like symptoms and fatigue usually disappear when the drug is discontinued.

Ongoing FDA Clinical Trials

Clinical trials are underway to test promising new potential treatments for CTCL including some biological therapies that use the body’s own immune system to fight the cancer and others that involve removing and treating the patient’s own bone marrow and returning it after chemotherapy.

Remember, though CTCL is not curable, most cases are treatable and are often not life-threatening. Your dermatologist at Dermatology and Skin Surgery will work closely with you to determine the best course of treatment to meet the challenges of this disease.