Peripheral T-Cell Lymphoma (PTCL)

Peripheral T-cell lymphoma (PTCL) is a rare group of aggressive (fast-growing) blood cancers. The cancer starts in lymphatic tissue, like your lymph nodes and spleen. But it can grow within and affect nearly every part of your body.

PTCL is an uncommon type of non-Hodgkin lymphoma. It accounts for only about 1 out of every 10 cases. This means that there’s still a lot that medical experts are learning about this condition.

Healthcare providers can successfully treat most of these lymphomas. But they often come back. Medical specialists are studying treatments that may help people live longer with this condition.

Types of peripheral T-cell lymphoma

The World Health Organization (WHO) recognizes more than 30 subtypes of PTCL. The three that are most common in the U.S. make up about 6 in 10 of all diagnoses. From most to least common, they are:

• Peripheral T-cell lymphoma, not otherwise specified: PTCL-NOS includes cases that don’t fit into the more specific subtypes.
• Anaplastic large cell lymphoma (ALCL): There are different forms of ALCL. They include primary cutaneous ALCL, which affects your skin. Another type — systemic ALCL — affects other organs in your lymph system.
• Angioimmunoblastic T-cell lymphoma (AITL): This type often causes problems with your immune system. As a result, your body may have trouble fighting infections. Or it may start attacking your own cells, leading to autoimmune diseases.

There are also rarer subtypes:

• Adult T-cell lymphoma/leukemia may affect your blood, lymph nodes or other lymph tissue.
• Enteropathy-associated T-cell lymphoma starts in your intestines.
• Extranodal natural killer/T-cell lymphoma, nasal type grows in your nose, sinuses or throat.
• Hepatosplenic T-cell lymphoma starts in your liver or spleen.
• Subcutaneous panniculitis-like lymphoma (SPTCL) starts in the bottom layer of your skin.

Symptoms of peripheral T-cell lymphoma

Each PTCL subtype has specific symptoms. But common ones include:

• Belly pain or swelling (a sign of an enlarged spleen)
• Drenching night sweats
• Persistent fatigue
• Skin rash
• Swollen lymph nodes
• Unexplained fever
• Unexplained weight loss

Peripheral T-cell lymphoma causes

Peripheral T-cell lymphomas happen when your T cells — a type of white blood cell — change (mutate). This makes them turn into cancerous cells that multiply uncontrollably.

Experts don’t know what makes T cells mutate in the first place.

Risk factors

Factors that may increase your risk of PTCL include:

• Where you live: Many subtypes of these lymphomas are more common in Asia (including China, Thailand and Japan), Africa and the Caribbean.
• Your age: The most common subtypes usually affect people over 60.
• Your health conditions: Some subtypes are linked to celiac disease, EBV and HTLV-1. Having a condition that causes a weakened immune system may increase your risk.

How doctors diagnose peripheral T-cell lymphoma

Your healthcare provider will consider your symptoms and check for signs of PTCL. Diagnosis may involve several tests, including:

• Blood tests: Your provider will check the health of your blood cells. They may test for substances that mean organ damage or viruses related to some types of PTCL.
• Imaging tests: A CT scan, MRI or PET scan can show tumors inside your body.
• Biopsies: Your provider may need to test tissue from your bones, lymph nodes or skin to check for cancer cells.
• Genetic tests: They’ll check cells for mutations that are common in peripheral T-cell lymphoma.

Stages of peripheral T-cell lymphoma

Healthcare providers use cancer staging systems to plan treatment. Providers stage PTCL based on factors like the subtype and where the cancerous T cells are. The stages are:

• Stage I: Cancerous T cells are in one lymph node, one cluster of lymph nodes or an organ.
• Stage II: Cancer affects two or more clusters of lymph nodes in the same area of your body.
• Stage III: Cancer affects lymph nodes in both the upper and lower parts of your body.
• Stage IV: Cancer affects lymph nodes and other organs like your lungs, liver or digestive tract.

How is peripheral T-cell lymphoma treated?

PTCL is so uncommon that healthcare providers are still learning which treatments work best. This is why your treatment plan will be customized to you. In some cases, the best treatment may be a clinical trial evaluating new PTCL treatments.

Common treatments include:

• Chemotherapy: You’ll take a combination of chemotherapy drugs.
• Radiation therapy: You may get this treatment along with chemotherapy.
• Targeted therapy: This treatment zeroes in on specific genetic mutations that turn healthy T cells into cancerous cells. Or it may target proteins that are part of cancerous T cells. Several drug types are being tested in clinical trials.
• Immunotherapy: This treatment is also more commonly available in clinical trials. It helps your immune system fight cancer.
• Stem cell transplant: A stem cell transplant may be the last step of treatment. Sometimes providers use it to treat PTCL that has come back after going away (relapse). There are different types of this treatment. The kind you need depends on your unique case.

When should I see my healthcare provider?

You’ll see your provider regularly to check on your response to treatment. They’ll also monitor any treatment side effects you’re experiencing.

But don’t wait for appointments to check in with your provider. Let them know if you’re having new side effects.

What’s the prognosis for peripheral T-cell lymphoma?

In some situations, treatment can cure PTCL. But most PTCLs don’t respond as well to standard treatments, like chemotherapy, as other types of lymphomas do. If it goes into remission (no signs or symptoms of cancer), it often comes back. At that point, your provider may suggest a different kind of treatment.

The five-year survival rate of peripheral T-cell lymphoma is 30% to 40%. This means that 3 to 4 out of every 10 people diagnosed are alive after five years. Still, your life expectancy depends on your unique diagnosis.

Is there anything I can do to feel better?

Living with lymphoma may mean rethinking your routines and support networks. Here are some suggestions that may help:

• Consider palliative care. This care can help you manage symptoms and treatment side effects. It can also help you deal with the emotional challenge of living with a serious illness.
• Find support. PTCLs are rare cancers. You may feel as if you’re alone in this. But you’re not. Talk to your healthcare team about support groups so you can connect with others in your situation.
• Take time for self-care. Self-care is essential. Talk to your healthcare team about ways to manage stress, eat right and stay active through treatment.