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Types of Cutaneous T-Cell Lymphoma
CTCL describes many different disorders with various symptoms, outcomes, and treatment considerations. The two most common types are mycosis fungoides and Sézary syndrome.
Mycosis fungoides: Mycosis fungoides is the most common type of CTCL, with approximately 16,000 to 20,000 cases across the United States, accounting for half of all CTCLs. The disease looks different in each patient, with skin symptoms that can appear as patches, plaques, or tumors. Patches are usually flat, possibly scaly, and look like a rash; plaques are thicker, raised, usually itchy lesions that are often mistaken for eczema, psoriasis, or dermatitis; and tumors are raised bumps, which may or may not ulcerate. It is possible to have more than one type of lesion.
A medical history, physical exam, and skin biopsy are essential for diagnosis. A physician will examine lymph nodes, order various blood tests, and may conduct other screening tests, such as a chest x-ray or a computed axial tomography (CAT) scan. Scans are usually not needed for those with the earliest stages of the disease. Mycosis fungoides is difficult to diagnose in its early stages because the symptoms and skin biopsy findings are similar to those of other skin conditions.
Sezary syndrome: Sezary syndrome is an advanced, variant form of mycosis fungoides, which is characterized by the presence of lymphoma cells in the blood. Extensive thin, red, itchy rashes usually cover over 80 percent of the body. In certain patients, patches and tumors appear. Patients may also experience changes in the nails, hair, or eyelids, or have enlarged lymph nodes.
Many of the same procedures used to diagnose and stage other types of cutaneous T-cell lymphomas are used in Sézary syndrome. In addition, a series of imaging tests may be needed to determine if the cancer has spread to the lymph nodes or other organs (although that uncommonly occurs). These tests may include a CAT scan, a positron emission tomography (PET) scan, and/or a magnetic resonance imaging (MRI) scan. A bone marrow biopsy may also be done, but is usually not necessary.
Causes of Cutaneous T-Cell Lymphoma
The causes of CTCL are unknown. Like other cancers, it’s not infectious and can't be passed on to other people.
Symptoms of Cutaneous T-Cell Lymphoma
The symptoms of CTCL will depend on the stage it is at when you are diagnosed. In the very early stage, CTCL can look like skin conditions such as eczema or psoriasis. There are red, rash-like patches on the skin. The skin may be scaly and the patches are sometimes raised (plaques). Itching is a common symptom. There may be one or more lumps (tumours) on the skin and some people may have swollen lymph nodes. In the advanced stage, large areas of skin are affected. The skin is very red, scaly and itchy, and it may be painful. It's often swollen and looks thicker in parts.
How Cutaneous T-Cell Lymphoma is Diagnosed?
The diagnosis is made by removing a small piece of affected skin (skin biopsy) and examining it under a microscope for abnormal cells. You can have this done under local anaesthetic. Your doctor will also carry out a full examination of your skin and lymph nodes. You will have blood tests taken to check if there are any abnormal lymphocytes present.
Some people may also have scans such as a CT (computerised tomography) scan or a MRI (magnetic resonance imaging) scan to check that the lymphoma hasn't spread. If your lymph nodes are swollen, you may have a biopsy or have them removed so they can be examined under a microscope for lymphoma cells. Occasionally a sample of the bone marrow may be taken (bone marrow biopsy) and examined under the microscope.
Treatment Options for Cutaneous T-Cell Lymphoma
The treatment you have will depend on the stage of the CTCL. Different treatments can be used and you may have a combination of them. Your specialist will discuss the treatment options with you. In early CTCL, treatments given directly to the skin are usually used. This includes steroid cream, chemotherapy cream (topical chemotherapy), PUVA or radiotherapy. These are explained below.
If CTCL is advanced or isn't responding to other treatments, systemic treatments are used. Chemotherapy (see below) or drugs called biological therapies are given directly into your bloodstream and can treat the whole body (systemic). You may also be given treatments directly to the skin.
Alemtuzumab (Mabcampath®): This drug is a monoclonal antibody. Monoclonal antibodies recognise and stick to certain proteins on the surface of some cancer cells. This can trigger the immune system to attack the cancer cells and cause them to destroy themselves. Alemtuzumab, usually called Campath, is generally given when CTCL has come back after other treatments have already been tried.
Bexarotene: Bexarotene (Targretin®) belongs to a group of drugs called retinoids. It can be taken daily as a capsule to treat CTCL that has come back after other treatments have been tried. Bexarotene can be given along with other treatments such as PUVA.
Chemotherapy: If CTCL is advanced or is not responding to other treatments, chemotherapy by injection into a vein, or as a tablet, may be given to help control it. Low doses of a drug called methotrexate or another drug called chlorambucil can be given as tablets to treat early disease. In advanced disease you may be given a single drug or sometimes a combination of drugs, usually by injection into a vein. Chemotherapy drugs that may be used are gemcitabine, pentostatin or liposomal doxorubicin.
Creams to the skin (topical treatments): Steroid cream applied to the skin may be the only treatment needed for early stage CTCL. Chemotherapy creams may be used. Chemotherapy is the use of anti-cancer (cytotoxic) drugs to destroy cancer cells. It is also given as a cream that can be applied to the affected skin. You'll need to make sure you only put the cream on the area where you've been told to apply it and you must wear gloves to protect your hands.
Your doctor, a nurse or a pharmacist will explain this to you. It's important to follow the instructions they give you carefully. Some people may have a reaction to the cream. Let your doctor know if you have any side effects, such as redness or a burning sensation in the area.
Extra-corporeal photopheresis (ECP): ECP is only used in advanced disease when the majority of the skin is affected and very red, and there are lymphoma cells in the blood. This treatment is only available in some specialist centres. ECP is a systemic (treats the whole body) type of PUVA treatment. Blood is removed from your body by connecting you to a special machine. The blood is then treated with ultraviolet light and drugs that become active when exposed to light. The treated blood is then returned to the body. Your doctor or nurse will give you more information about this treatment.
Interferon: Interferon is a protein that occurs naturally in the body. It is sometimes injected just under the skin (subcutaneously) to boost the body’s own immune system to control the lymphoma. It may be given on its own or with other treatments, such as PUVA or bexarotene. The most common side effects of interferon are tiredness, aches and pains, fever and a low mood.
PUVA treatment: PUVA (sometimes known as photochemotherapy) is an effective treatment for early CTCL, especially for treating plaques (raised red patches). It involves taking a drug called psoralen (P), which makes your skin sensitive to ultraviolet light (UVA). Once the drug has had time to collect in your skin, you go into an enclosed air-conditioned cabinet to have the ultraviolet light treatment.
You may have this done a few times a week, with treatments reducing as skin symptoms improve. Treatment with PUVA is sometimes continued when the symptoms have gone away. This is done to help keep CTCL in remission (when there are no signs of the disease).
This treatment tans the skin and can cause early ageing of the skin. Sometimes the skin may become red, like mild sunburn. Over the period of your treatment, you'll need to be careful not to expose your skin to extra ultraviolet light from the sun. Exposure to ultraviolet light can increase the risk of developing other types of skin cancer. The number of treatments you have with PUVA is limited to reduce the risk of this happening, and your skin is carefully monitored. Your doctor or specialist nurse can discuss this with you further. We have more information on taking care of your skin in the sun.
Psoralen makes the eyes more sensitive to ultraviolet light, so you will need to wear protective glasses for around 12 hours from the time the drug is taken. The psoralen tablets can make you feel sick. If this happens you can be given anti-sickness (anti-emetic) drugs or it may be possible to be prescribed a different tablet. Other possible side effects of PUVA include itching, dizziness and headaches.
Radiotherapy: Radiotherapy is the use of high-energy rays to destroy cancer cells. It's an effective treatment for early CTCL, when there are small areas of affected skin in the one area. It can also be used to ease skin symptoms when CTCL is advanced. The side effects are redness of the skin and hair loss in the area being treated.
Total electron beam therapy (EBT): This is a type of radiotherapy. It can be used to treat larger areas of CTCL, that haven’t spread below the skin surface. The treatment can be repeated if necessary. It's only available at some specialist centres.
The side effects are mainly skin changes and hair loss in the area of skin that's being treated. Sweat glands in treated areas may no longer work. The extent of side effects will depend on the size of the area treated.
UVB therapy: UVB light (ultraviolet light from wavelength B) can help slow down the growth of skin cells and may be used to treat CTCL. The treatment is delivered in a similar way to PUVA, using an air-conditioned cabinet containing ultraviolet lights. However, you don’t need to take a drug to make your skin more sensitive to light. Treatment may be given a few times a week.
UVB therapy causes similar skin side effects to PUVA. Because ultraviolet light increases the risk of other types of skin cancer, you will also need to avoid exposing the skin to extra UV light from the sun. Your doctor or specialist nurse can discuss this with you further.
Treatments Under Investigation
Many treatments at various stages of drug development are currently being tested in clinical trials and for various stages of CTCL, including everolimus (Afinitor), lenalidomide (Revlimid), brentuximab vedotin (Adcetris), panobinostat, forodesine, APO866, KW0761, and others. It is critical to remember that today's scientific research is continuously evolving. Treatment options may change as new treatments are discovered and current treatments are improved. Therefore, it is important that patients check with LRF or with their physician for any treatment updates that may have recently emerged.