Cutaneous T-Cell Lymphoma
What is cutaneous T-cell lymphoma?
Cutaneous T-cell lymphoma is a group of rare blood cancers that affects the largest organ in your body — your skin. These cancers cause symptoms like rash, very itchy skin (pruritus) or other skin issues that may look and feel like common skin disorders. Most cutaneous T-cell lymphomas grow very slowly and aren’t life-threatening, but some people may develop serious forms of the condition. Healthcare providers have treatments to ease symptoms, but they can’t cure the lymphomas.
How do these lymphomas affect my body?
Cutaneous T-cell lymphomas are part of a larger group of diseases called non-Hodgkin lymphoma. Non-Hodgkin lymphomas are cancers that start in certain white blood cells called lymphocytes. Two types of lymphocytes — CD4 and CD8 — help regulate your immune system. In cutaneous T-cell lymphoma, T-cell lymphocytes mutate, becoming cancerous cells that multiply uncontrollably.
Cutaneous T-cell lymphomas can look and feel like common skin problems like psoriasis, eczema or even an allergic reaction. Many people have symptoms for years before obtaining a diagnosis. The two most common subtype of cutaneous T-cell lymphoma are mycosis fungoides and Sézary syndrome. Each subtype may affect your body in different ways:
- Mycosis fungoides: This is the most common type of cutaneous T-cell lymphoma. It accounts for 50% of all cases. It’s a slow-growing cancer. Typically, cancerous cells are only in your skin. About 10% of the time, however, cancerous cells may spread from your skin into your lymph nodes and/or internal organs such as your liver, spleen or digestive system, and cause life-threatening medical complications.
- Sézary syndrome: This is the second most common type of cutaneous T-cell lymphoma. It represents about 15 % of all cases. In Sézary syndrome, cancerous lymphocytes (Sézary cells) are in your skin and your bloodstream. People with this syndrome may have widespread redness on their skin. Sometimes the condition may involve your lymph nodes and other areas of your body. Sézary syndrome is an aggressive disease, meaning it spreads fast. Treatment can help manage the effects of the disease, but there’s no cure.
Is cutaneous T-cell lymphoma a kind of skin cancer?
Cutaneous T-cell lymphomas are blood cancers that affect your skin, but they’re not skin cancers. The difference lies in where cancer starts. In cutaneous T-cell lymphoma, white blood cells called lymphocytes mutate, becoming cancerous cells that multiply and cause symptoms like widespread redness, itchy rashes, large patches of raised skin or small firm lumps (nodules). Skin cancer like basal cell carcinoma happens when skin (epidermal ) cells mutate, becoming abnormal skin cells that multiply and form tumors on or under your skin.
Are cutaneous T-cell lymphomas serious illnesses?
They can be serious illnesses but that doesn’t happen very often. For example, mycosis fungoides, the most common subtype, mostly affect your skin, grows very slowly and is easy to treat.
Who does it affect?
Cutaneous T-cell lymphomas are twice as likely to affect men and people designated male at birth than women and people designated female at birth. These lymphomas typically affect people between the ages of age 40 to 60. People who are Black are more likely to develop these conditions than people who are white or Asian American.
How common are cutaneous T-cell lymphomas?
These are rare illnesses. Healthcare providers estimate mycosis fungoides, the most common form of cutaneous T-cell lymphoma, affects 1 in 1 million people in the United States.
Symptoms and Causes
What are cutaneous T-cell lymphoma symptoms?
Symptoms vary depending on the disease type and phase. For example, mycosis fungoides symptoms may begin as red rashes or red scaly patches on your body. Sézary syndrome also affects your skin but can spread to your bloodstream.
What causes cutaneous T-cell lymphoma?
Healthcare providers and researchers don’t know exactly what causes cutaneous T-cell lymphomas happen. Here are two possibilities:
- Acquired genetic mutations: Researchers have found some gene changes that cause these conditions. They’re still studying why certain genes change.
- Infections: Your immune system goes into overdrive when you have an infection. Your bone marrow reacts by creating more lymphocytes more quickly. Just like any production line, speeding up the process may lead to mistakes. In this case, the mistakes stem from DNA mutations that affect key genes in lymphocytes that eventually cause lymphoma. Researchers are investigating possible links between infections and cutaneous T-cell lymphoma.
Diagnosis and Tests
How do healthcare providers diagnose cutaneous T-cell lymphoma?
Your healthcare provider will review your medical history and do a physical examination. They’ll focus on any patches or plaques on your skin. They may ask if you have allergies.
What tests do providers use to diagnose this condition?
- Blood tests: Your doctor will check your blood to look at your blood counts, electrolytes, and kidney and liver function. They’ll do special tests to look for signs of lymphoma in your blood and count any lymphoma cells they find.
- Biopsies: Healthcare providers do skin biopsies to establish a diagnosis. They may need to do several skin biopsies to accurately diagnose cutaneous T-cell lymphoma. They may also do lymph node biopsies and biopsies of any other parts of your body that may be affected by cutaneous T-cell lymphoma.
- Imaging studies: Healthcare providers may use computed tomography (CT) scans and positron emission tomography (PET) scans to examine your lymph nodes and other organs.
Staging for mycosis fungoides and Sézary syndrome
Healthcare providers use cancer-staging systems to plan treatments and develop prognoses or expected outcomes. Mycosis fungoides and Sézary syndrome stages range from I to IV. Each stage has four sub-categories from 1-4. Providers also use a staging system known as TNMB, based on four factors:
- Tumor size (T): This measures how much skin is affected by the condition.
- Lymph nodes (N): This measures the amount of lymphoma in your lymph nodes.
- Metastasis (M): This measures whether lymphoma has spread from your skin to other organs.
- Blood (B): This measures the amount of lymphoma cells in your blood.
Management and Treatment
How do healthcare providers treat cutaneous T-cell lymphoma?
- Skin-directed treatments: Healthcare providers may use topical ointments, creams, or light treatments for early-stage cutaneous T-cell lymphoma.
- Phototherapy: This treatment is used for early-stage disease. In phototherapy, healthcare providers use ultraviolet A(UV-A) or B (UV-B) light to treat early-stage disease. They may combine UV-A with psoralen in what's referred to as PUVA treatment. After taking the medication, healthcare providers expose the affected areas of your skin to UV light. The light activates the medication and kills cancer cells.
- Extracorporeal photophoresis (ECP): This treatment involves ultraviolet light and psoralen. Providers take some of your blood so they can obtain white blood cells. They mix those cells with psoralen. This makes cancerous cells vulnerable to ultraviolet (UV) light. These cells are then exposed to UV light, killing the cancer cells. They then return the treated white blood cells to your bloodstream.
- Radiation therapy: Providers target cancer cells with X-rays or other radiation sources, slowing cancer cell growth or killing the cells.
- Immunotherapy: This treatment boosts your immune system with lab-made substances or your body’s substances. Immunotherapy helps slow cancer cell growth or eliminate cancer cells.
- Chemotherapy: You may take chemotherapy as pills, via intravenous injection (IV) or as gels, creams or lotions that are directly applied to your skin’s affected areas.
Can I prevent cutaneous T-cell lymphoma?
Healthcare providers and researchers aren’t sure why these conditions happen. They do see some connections between conditions like infections or medical treatment that may weaken your immune system. Ways you can protect your immune system include:
- Make sure you’re up-to-date on vaccines.
- Try to quit smoking.
- Maintain a weight that’s healthy for you.
- Eat a healthy diet that includes lots of fruits and vegetables.
- Avoid alcohol or use it only in moderation.
- Get enough sleep.
- Exercise regularly.
- Wash your hands often.
- Try to stress less and focus on mind/body wellness.
Outlook / Prognosis
Can cutaneous T-cell lymphomas be cured?
Healthcare providers can’t cure these skin lymphomas. They can treat the conditions with therapies that ease or eliminate symptoms and slow down lymphoma growth. Many times people who receive treatment for this condition will live for many years.
What is the survival rate for cutaneous T-cell lymphoma?
Many things factor into estimating a survival rate. If you have cutaneous T-cell lymphoma, your healthcare provider is your best resource for information about your situation
How do I take care of myself?
Cutaneous T-cell lymphoma is cancer that affects your skin, making it dry, itchy and scaly. While treatment slows cancer growth and eases symptoms, some treatments may irritate already aggravated skin. Here are some suggestions that may help:
- Keep your skin moist: Use creams or ointments after you bathe or shower to lock in moisture and protect your skin. You may want to re-apply creams or ointments throughout your day and before you go to bed. In addition to protecting your skin, moisture combats dryness that may make your skin flake, helps with itchiness and may protect your skin from infection.
- Protect your skin from irritation: If you have a type of cutaneous T-cell lymphoma, your skin is more vulnerable. Everyday activities like being in the sun, using certain laundry detergents and soaps or even wearing certain clothes can affect your already-tender skin. Look for fragrance-free detergents and body soaps. Wear clothing that protects your skin from the sun and use sunscreen when going outside. Wear loose-fitting clothes that let your skin breathe.
- Don’t scratch that itch: For many people, incessantly itchy skin is a serious problem affecting their quality of life. Scratching itchy skin can cause infections. Cold compresses (think of bags of frozen vegetables), oatmeal baths and antihistamines may help ease itchy skin.
When should I see my healthcare provider?
You should discuss with your healthcare provider about how often you need to see them. They’ll decide how often you need to have skin examinations and/or blood tests. Always contact your healthcare provider if:
- You notice any changes in the parts of your skin affected by cutaneous T-cell lymphoma.
- You think your skin has become infected or you have a fever.
A note from Cleveland Clinic
Cutaneous T-cell lymphoma is a group of rare blood cancers that affect your skin. Most cutaneous T-cell lymphomas grow very slowly and aren’t life-threatening. Dealing with cutaneous T-cell lymphoma can be challenging for people and healthcare providers alike. It may take you a while to ask your provider about a persistent rash. It may take your provider a while to confirm that rash isn’t one of several common skin problems. After that, it may take several tests before they can confirm you have a form of cutaneous T-cell lymphoma. Once your healthcare providers diagnose cutaneous T-cell lymphoma, they have several treatments to ease your symptoms.
Cleveland Clinic is a non-profit academic medical center. Advertising on our site helps support our mission. We do not endorse non-Cleveland Clinic products or services. Policy