What is cutaneous T-cell lymphoma?
Cutaneous T-cell lymphoma (CTCL) is a type of cancer of the T-lymphocytes (white blood cells) that affects the skin and the blood. Occasionally, it also involves the lymph nodes and internal organs.

The malignant T-cells are attracted to the skin and can appear anywhere on the body surface. If it is mild, there will only be a rash; but if it is more severe, thick lesions called plaques can form. In some instances, the skin becomes red all over.

What is the progression of CTCL?
The course of CTCL is unpredictable. Some patients progress slowly, some rapidly, and some not at all. Most patients will only experience skin symptoms without serious complications. About 10% of people diagnosed with CTCL will experience a progression with lymph node and internal involvement or other serious complications. Most patients live normal lives while they treat their disease, and some are able to remain in remission for long periods of time.

Is there a cure?
While there is no cure, research is ongoing. Patients diagnosed early (disease involving less than 10% of the body) will live a normal life expectancy. If you have symptoms, it is best to see your dermatologist.

Causes of CTCL
CTCL is a rare disease -- five to 10 persons per million are affected. The cause of CTCL remains unknown, but research continues. CTCL is not contagious and is not inherited. Men are affected more than women, and it is more common after the age of 50.

Types of CTCL
There are many types of CTCL, all of which differ in appearance, progression, and treatment. The two main types are mycosis fungoides and Szary syndrome.

Mycosis Fungoides - This is the most common type of CTCL, which primarily affects the skin. Generally, it has a slow course and often remains confined to the skin. Mycosis fungoides have three phases: patch, plaque, and tumor. The patient may have one or all of these phases, which can appear anywhere on the skin. Patches are usually flat, red, and scaly. They are often mistaken for eczema or dermatitis because they may itch. Plaques are thicker, raised lesions. Tumors are larger lesions that can ulcerate and become huge and mushroom shaped (fungoides). The disease is NOT a fungal infection.

Szary Syndrome - This is the advanced form of mycosis fungoides and affects the blood. It consists of red skin, a large number of tumor cells found in the blood (leukemia), and larger-than-normal lymph nodes. Often referred to as the "red-man disease," patients with Szary syndrome often are red from head to toe and complain that their skin is hot, sore, and itchy. There may be intense skin flaking, itching and burning; loss of hair; thickening of the palms, fingernails, and soles; drooping eyelids; loss of eyelashes; and difficulty closing the eyes.

Diagnosis
CTCL is not an easy disease to diagnose. It may take years to make a diagnosis. Dermatologists diagnose CTCL from the patient's medical history, performing a physical examination, and obtaining blood tests and skin biopsies. Many skin biopsies may be needed in order to make the correct diagnosis.

Treatment
The goal of treatment is to control symptoms such as itching and burning and to make the patches and skin tumors go away. In Szary syndrome, treatment reduces skin redness and reduces the number of abnormal lymphocytes in the blood.

Treatment is based on the type of CTCL, patient's health, age and lifestyle and the extent of the disease. Different treatments include application of creams and ointments to the skin, oral medication, light therapies (phototherapy), interferon injections, and radiation. Different types of biological therapies that use the body's own immune system to fight the cancer are being tested in clinical trials.

Topicals

Cortisone (corticosteroid) Cream - Cortisone is a drug that reduces inflammation. Cortisone creams, ointments, gels, and lotions temporarily control skin inflammation in many patients with CTCL. Generally, lower strength cortisone preparations are used on sensitive areas of the body such as the groin, armpits, and face. Stronger preparations are usually needed to control affected skin elsewhere on the body. Side effects of the stronger cortisone preparations include thinning of the skin, dilated blood vessels, bruising, and skin color changes. If creams are stopped too quickly, the disease may get worse. CTCL may become resistant to cortisone creams with time.

Nitrogen Mustard Ointment and Liquid - Nitrogen mustard ointment and liquid is a type of topical chemotherapy that may clear the skin temporarily and control CTCL. Patients use gloves to apply nitrogen mustard once daily. The face, groin, and armpits are sensitive, so patients should ask their dermatologist whether these areas should be avoided. A possible side effect may be an allergic reaction to nitrogen mustard, which involves skin irritation.

Retinoids (gel) - Also known as bexarotene, retinoids are derivatives of vitamin A. Bexarotene can be used as a gel or taken orally. Bexarotene gel was approved by the FDA in 2000 for patients with early-stage CTCL. When applied to the skin, it acts by interfering with the growth of cells of the tumor. Side effects of taking bexarotene gel may be skin rash, redness, and itching.

Oral

Corticosteroids - This is a group of drugs that have powerful anti-inflammatory properties. A corticosteroid like prednisone is common and is usually used only in severe cases of CTCL. It can be used alone or in combination with other treatments to control CTCL.

Side effects from taking corticosteroids over a long period of time include weight gain, development of a round face, increased blood sugar levels (diabetes), and thinning of the bones. A dermatologist will watch for side effects.

Retinoids (capsule) - The oral form of bexarotene gained FDA approval in 1999 for patients with advanced-stage CTCL or for patients who have not responded well to other therapies. The capsule acts by selecting cancerous T-cells and causing apoptosis (cell death). The capsules are taken every day and are easily tolerated.

Side effects may include an allergic reaction, headaches, fatigue, weakness, swelling, rash, dry skin, nausea, elevation of the blood fat (triglycerides) and cholesterol, decreased thyroid function, and changes in liver function. The dermatologist will monitor you with regular blood tests for side effects. Medication may be needed to control high fat levels in the blood.

Methotrexate - This is an oral anticancer drug that is used to control CTCL. Side effects include upset stomach, nausea, mouth ulcers, and dizziness. Liver function is monitored as well.

Systemic Chemotherapy

These medications kill cancer cells intravenously. Chemotherapy given in this way is called systemic treatment because the drug enters the bloodstream and travels through the body killing cancer cells. Many different types of drugs are used for systemic chemotherapy.

Fusion Protein – This is an immune system called interleukin-2 that is fused with a toxin (diphtheria). Fusion protein works by seeking out and attaching to receptors for IL-2 found on malignant T-cells. This allows the toxin to be taken inside and kills the malignant T-cells. Fusion protein has been approved for recurrent CTCL patients in all stages of the disease.

Side effects of chemotherapy depend on the type of drug being used.

Light Therapies

Ultraviolet light B (UVB) or Narrow-band UVB – This slows the rapid growth of skin cells and is safe and effective under a doctor's care. Light boxes with full-body exposure are used to deliver ultraviolet rays that can treat CTCL.

PUVA - The name "PUVA" stands for "psoralen," (the drug) and the term "UVA," the specific type of ultraviolet light. After psoralen pills are taken, a carefully measured amount of UVA light is delivered to the patient in a light box. Treatments are usually delivered three times a week, and it may take several months of treatment until there is improvement. The frequency of PUVA treatments may be decreased and a maintenance regime set up when the patient is clear. Psoralen temporarily remains in the lens of the eye; therefore, patients must wear UVA-blocking sunglasses on the days of treatment.

Extracorporeal Photopheresis (ECP) - The term "extracorporeal" means "outside the body," and "photopheresis" comes from the Greek words "photo" (meaning "light") and "aphairesis" (meaning "removal"). During treatment, blood is taken from a vein and circulated through a machine where it is sensitized with psoralen, then exposed to ultraviolet light, and then returned to the body. This process causes selective destruction of the cancerous cells in the blood. To receive treatment, patients usually visit a medical center for two days once a month.

Side effects of all light therapies include burning of the skin (like a sun burn), premature aging, freckling, and skin cancer.

Radiation Therapy

X-ray Therapy - Spot radiation is sometimes used to focus on the affected area in the skin in an effort to kill cancerous cells. Another type of radiation is directed at the whole body – this is called total body irradiation, or TSEB (total skin electron beam).

Side effects of radiation therapy include inflammation of the skin, hair and nail loss, and lack of energy.

Interferon - This medication is used to control tumor growth. It is given by injection under the skin three to five times a week. Injections can be given by the patients themselves, by a person at home, or by a dermatologist.

Side effects include flu-like symptoms, fatigue and lack of energy. Side effects usually disappear when the drug is discontinued.

Ongoing FDA Clinical Trials

Biological Therapy – This type of therapy tries to get the body to fight the cancer. It uses materials made by the body to restore the body's natural defenses against the disease. Although various new biological drug modifiers are being tested, they are not yet approved by the FDA.

Also in research is a type of bone marrow transplant called autologous bone marrow transplant, in which bone marrow is taken from the patient and treated with drugs to kill any cancer. The marrow is then frozen while the patient undergoes chemotherapy and is given back to the patient to replace what was destroyed once the patient is finished with the chemotherapy treatment.