Cutaneous T-Cell Lymphoma (CTCL)

What is cutaneous T-cell lymphoma (CTCL)?

Cutaneous T-cell lymphoma (CTCL) is a group of rare blood cancers that affects the largest organ in your body — your skin. CTCLs can look and feel like common skin conditions like psoriasis, eczema or even allergic reactions. Many people have symptoms like skin rash, itchiness or discoloration for years before obtaining a diagnosis.

CTCLs are part of a larger group of cancers called non-Hodgkin lymphoma, which start in certain white blood cells called lymphocytes.

Many people treated for CTCL go into remission, returning to their daily routines without much disruption. Still, a cancer diagnosis can take a toll on your mental and emotional health. Your healthcare team is here to help.

Types of cutaneous T-cell lymphoma

There are many types of cutaneous T-cell lymphoma. But the most common are mycosis fungoides and Sézary syndrome:

• Mycosis fungoides: This slow-growing cancer accounts for about half of all CTCL cases. It happens when T cells (T lymphocytes) become cancerous. The cancerous cells usually don’t spread beyond your skin. But a few might find their way to your lymph nodes and bloodstream. When cancerous T-cells circulate in your blood, providers call them Sézary cells.
• Sézary syndrome: With this fast-growing CTCL, you have large numbers of Sézary cells in your skin and your bloodstream. Mycosis fungoides can turn into Sézary syndrome. People with this condition often have widespread skin discoloration.

How common is the condition?

Cutaneous T-cell lymphomas are rare. Healthcare providers estimate mycosis fungoides, the most common form of CTCL, affects 1 in 1 million people in the United States.

What are the symptoms of CTCL?

Symptoms of cutaneous T-cell lymphoma include:

• Bumps on your skin that might break open.
• Hair loss.
• Itchy, rash-like discoloration all over your body.
• Patches of skin discoloration.
• Raised skin rash that might be flaky or itchy.
• Swollen lymph nodes.
• Thickened skin on the palms of your hands and the soles of your feet.

What causes cutaneous T-cell lymphoma?

In cutaneous T-cell lymphoma, T lymphocytes mutate (change) into cancerous cells that multiply uncontrollably. Healthcare providers don’t know exactly why CTCLs happen, or why some people are more likely than others to get them. Here are two possibilities:

• Genetic variant (mutation): Researchers have identified some gene changes that might cause these conditions.
• 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 your lymphocytes. This eventually causes lymphoma.

Risk factors of CTCL

You’re more likely to develop cutaneous T-cell lymphoma if you:

• Are Black.
• Are over 50.
• Have a weakened immune system.
• Were assigned male at birth (AMAB).

How is cutaneous T-cell lymphoma diagnosed?

A healthcare provider will review your medical history and do a physical examination, focusing on any patches or plaques (thickened areas) on your skin. They’ll ask you about your symptoms and review your health history, including any prior or current health conditions.

Tests used to diagnose the condition

Your healthcare provider will need to run tests to confirm a cutaneous T-cell lymphoma diagnosis. These tests may include:

• Blood tests.
• Imaging tests like CT scans or PET scans.
• Lymph node biopsy.
• Skin biopsy.

Stages of cutaneous T-cell lymphoma

Healthcare providers use staging systems to categorize, diagnose and treat cancers. The TNM classification system is the most common for staging CTCLs. This system describes:

• The primary tumor, or where the cancer started (T).
• Whether the cancer has spread to nearby lymph nodes (N).
• Whether the cancer has spread (metastasized) to distant areas of your body (M).

The TNM system uses numbers and letters to stage cutaneous T-cell lymphoma:

How is cutaneous T-cell lymphoma treated?

Healthcare providers use several methods to treat CTCL, including:

• Steroid creams or ointments.
• Light therapy.
• Extracorporeal photopheresis (ECP).
• Radiation therapy.
• Immunotherapy.
• Chemotherapy.

Treatment is different for everyone. Your provider will let you know which options they recommend in your situation.

Can cutaneous T-cell lymphoma be prevented?

Most people who have CTCL have no changeable risk factors. So, there’s nothing you can do to prevent it. But researchers continue to study why these conditions happen.

How can I lower my risk?

Even though you can’t prevent cutaneous T-cell lymphoma, a weakened immune system could make you more susceptible to CTCLs and all types of cancer. Here are some ideas for keeping your immune system strong:

• Eat lots of fruits and vegetables.
• Exercise regularly.
• Get enough good-quality sleep.
• Limit drinks containing alcohol.
• Maintain a weight that’s healthy for you.
• Manage stress with meditation or other mindfulness activities.
• Stay up to date on vaccines.
• Try to quit smoking.
• Wash your hands often.

What’s the prognosis for people with cutaneous T-cell lymphoma?

Most CTCLs grow very slowly and aren’t life-threatening. But some people develop serious forms of the condition. Healthcare providers can’t cure CTCLs, but they can successfully manage symptoms with treatment.

But like all cancers, CTCLs can be fatal in their advanced stages. The sooner you start treatment, the better. That’s why it’s so important to tell a provider whenever you notice changes in your skin.

What is the survival rate for cutaneous T-cell lymphoma?

The overall 10-year survival rate for people with early-stage (Stage I or II) CTCL is 90%. That means that 9 out of 10 people with this condition are still alive 10 years later. People with late-stage CTCL (Stage III or IV) have an overall 10-year survival rate of 53%.

Survival rates are estimates. They can’t tell you how long you’ll live or how you’ll respond to treatment. To learn more about survival rates and what they mean for you, ask your healthcare provider.

How do I take care of myself?

Cutaneous T-cell lymphoma 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 makes your skin flake, helps with itchiness and may shield your skin from infection.
• Protect your skin from irritation. If you have 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 fabrics can irritate your 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 issue affecting their quality of life. Scratching can break open your skin and allow bacteria to get inside. This can lead to infections. Cold compresses (think bags of frozen vegetables), oatmeal baths and antihistamines may help soothe itchy skin.

When should I see my healthcare provider?

Your healthcare provider will want to see you for regular checkups. Ask them how often you should see them for blood tests or skin exams.

Always contact your healthcare provider if you:

• Notice any skin changes.
• Think you might have a skin infection.
• Develop a fever over 100.4 degrees Fahrenheit (38 degrees Celsius).