Peripheral T-cell Lymphoma (PTCL)

What is peripheral T-cell lymphoma (PTCL)?

Peripheral T-cell lymphoma (PTCL) isn’t a single disease. The term refers to a group of aggressive (fast growing) blood cancers that affect your lymphatic system and may spread (metastasize) to other areas of your body. Peripheral T-cell lymphomas are a form of non-Hodgkin lymphoma. They can affect nearly every part of your body and cause many different symptoms. Healthcare providers can successfully treat most subtypes, but the conditions often come back (recur). Medical researchers are studying potential treatments that may help people to live longer with PTCL.

Are peripheral T-cell lymphomas common?

No, they aren’t. One global study concluded PTCLs affect 2 in 100,000 people worldwide, and they make up about 10% of cases of non-Hodgkin lymphoma. The lymphomas are more common in Asia, Africa and the Caribbean than in the United States. They typically affect people ages 60 and older, but children and young adults may develop certain types of these lymphomas.

What are the types of peripheral T-cell lymphoma?

The World Health Organization (WHO) recognizes more than 20 PTCL subtypes. Nearly all PTCL subtypes have distinct genetic markers and other characteristics. According to one global study, the most common PTCL subtypes are:

• Peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS):This is the most common subtype. PTCL isn’t a specifically defined subtype. Instead, it’s a group of conditions that represent cases that don’t fit well into one of the more specifically defined subtypes. Approximately 30% of all peripheral T-cell lymphoma cases are PTCL-NOS. This subtype affects your lymph nodes, bone marrow, spleen or liver.
• Angioimmunoblastic T-cell lymphoma (AITL): This subtype represents between 15% and 30% of PTCL cases worldwide. It affects your lymph nodes, bone marrow, spleen or liver.
• 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 your lymph nodes, skin and other organs. Some cases of systemic ALCL are further categorized by changes in a specific gene, called anaplastic lymphoma kinase (ALK). ALCL represents approximately 15% of PTCLs.
• Extranodal natural killer/T-cell lymphoma, nasal type: This PTCL subtype commonly grows in the tissues of your nose, sinus cavities and upper throat, but it may also spread to your skin, digestive tract and other organs. Approximately 10% of PTCLs are this subtype.
• Intestinal T-cell lymphomas, which account for about 6% of PTCLs. This subtype affects your digestive system and includes enteropathy-associated T-cell lymphoma (EATL) and monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL).
• Adult T-cell lymphoma/leukemia, which may affect your skin and bones. There are four subtypes of ATLL: acute, lymphoma, chronic and smoldering. Acute and lymphoma subtypes are aggressive (fast growing) forms of ATLL. Chronic and smoldering are less aggressive. Each ATLL subtype affects different areas of your body.

What are symptoms of peripheral T-cell lymphomas?

While each PTCL subtype has specific symptoms, some common symptoms include:

• Swollen lymph nodes: You may have painless swelling in your neck, armpits or groin.
• Unexplained weight loss: This is losing body weight without trying. Losing 10% of your total body weight over six months is a sign of unexplained weight loss.
• Belly (abdominal) pain or swelling: This may happen if your spleen becomes enlarged.
• Persistent fatigue: This is feeling much more tired than usual for at least several days and for no apparent reason.
• Unexplained fever: Often, fevers are signs your body is fighting an infection. A fever that stays above 103 degrees Fahrenheit (39.5 degrees Celsius) for two or more hours after home treatment or lasts longer than two days may be a sign of a serious problem.

Specific subtypes may have other symptoms.

Peripheral T-cell lymphoma, not otherwise specified

Symptoms other than ones common to PTCL include:

• Anemia (low red blood cell levels).
• Thrombocytopenia (low platelet levels).
• Itchy red patches on your skin.
• Chest pain or shortness of breath (dyspnea) if there’s cancer in your chest.

Angioimmunoblastic T-cell lymphoma

Symptoms other than ones common to PTCL include:

• Night sweats.
• Skin rash.
• Autoimmune disorders like autoimmune hemolytic anemia.

Anaplastic large cell lymphoma

Primary cutaneous ALCL

Symptoms include:

• Abnormal reddish or reddish-brown growths on your skin that grow over time.
• Large, raised bumps that itch.
• Growths that become wounds and scab over.

Systemic ALCL

Systemic ALCL causes common PTCL symptoms.

Extranodal natural killer/T-cell lymphoma, nasal type

Symptoms include:

• Congestion (your nose feels blocked up).
• Nosebleeds.
• Crust in your nose.
• Painful swelling that affects your face.
• Eye issues like “weepy” eyes or eye pain.

Adult T-cell lymphoma/leukemia

The four ATLL subtypes have common PTCL symptoms.

What causes peripheral T-cell lymphomas?

Peripheral T-cell lymphomas happen when your T cells mutate and become cancerous cells. T cells are white blood cells that help defend your body from intruders like germs. When T cells mutate, they turn into cancerous cells that multiply uncontrollably. As they multiply, they build up in your lymph nodes, spleen, liver and other organs and create cancerous tumors.

Experts don’t know exactly what makes T cells mutate to cause peripheral T-cell lymphoma. But research links them to certain medical conditions. For example, one global study showed having celiac disease may increase your risk of developing several types of PTCL, including anaplastic large cell lymphoma. For another example, being infected with Epstein-Barr virus may increase your risk of developing extranodal natural killer/T-cell lymphoma. Having the human T-cell lymphotropic virus Type 1 (HTLV-1) may increase your risk of acute T-cell lymphoma/leukemia.

How is peripheral T-cell lymphoma diagnosed?

Healthcare providers may need to do several different tests to find the specific PTCL that’s causing health issues. You may need blood tests, imaging tests, biopsies and genetic tests when pathologists study cancerous cells’ genetic makeup.

Blood tests

Providers may test your blood for viruses linked to peripheral T-cell lymphomas. Blood tests may include:

• Complete blood count (CBC).
• Comprehensive metabolic panel (CMP).
• Lactate dehydrogenase (LDH) test.
• Hepatitis B and Hepatitis C tests.
• Human immunodeficiency virus (HIV) test.
• Human T-cell lymphotropic virus Type 1 (HTLV-Type 1) tests.

Imaging tests

Imaging tests give healthcare providers information about what’s going on inside your body, including tumors.

• Computed tomography (CT) scan.
• Positron emission tomography (PET) scan.
• Magnetic resonance imaging (MRI) scan.

Biopsies

• Lymph node biopsy.
• Skin biopsy.
• Bone marrow biopsy.

What are the stages of peripheral T-cell lymphoma?

Providers use cancer staging systems to develop treatment plans and prognoses, or what you may expect from treatment. They set cancer stages based on factors like PTCL type and where the cancerous T cells are growing. Peripheral T-cell lymphoma stages are:

• Stage I: Cancerous T cells in one lymph node or in one cluster of lymph nodes.
• 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 or your digestive tract.

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

There’s no single treatment for the many PTCL subtypes, and treatment may vary depending on cancer stage. Common treatments may include:

• Chemotherapy, combining different types of chemotherapy drugs.
• Radiation therapy, which providers may use along with chemotherapy.
• Targeted therapy, which targets specific genetic changes or mutations that turn healthy cells into cancerous cells.
• Chemotherapy and allogeneic stem cell transplantation to treat PTCL that doesn’t respond to chemotherapy and radiation therapy or that’s come back after treatment.

You may want to consider taking part in a clinical trial evaluating PTCL treatments.

What are common treatment side effects?

Most cancer treatments may cause side effects. For example, chemotherapy and radiation therapy side effects may include:

• Fatigue.
• Chemotherapy brain fog.
• Nausea and vomiting.

Can peripheral T-cell lymphomas be prevented?

No, they can’t. These lymphomas happen when T cells mutate and become cancerous cells. There’s nothing you can do to prevent that.

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

That depends on the type and stage of PTCL and whether treatment has put cancer into remission. Remission means you don’t have symptoms and tests don’t find signs of cancer. In some situations, standard treatment can cure PTCL. However, most peripheral T-cell lymphomas come back, which means you’ll need additional treatment or a different kind of treatment.

It’s understandable that you want to know what you may expect from treatment. Consider your healthcare provider your best resource for information about your situation.

How do I take care of myself?

Peripheral T-cell lymphomas are rare, fast-growing cancers. If you have a form of PTCL, here are some suggestions that may help you live with the condition:

• Consider palliative care: This care can help you manage symptoms and treatment side effects. More than that, your palliative care team can support you as 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 the only person who knows what you’re going through. You don’t have to face cancer alone. Talk to your healthcare team about support groups so you can connect with others in your situation.
• Take time for self-care: Cancer is stressful. Self-care is an important part of living with PTCL. Talk to your healthcare team about ways to manage stress and how to develop a nutritious diet to keep you strong through treatment.

What questions should I ask my healthcare provider?

• What type of peripheral T-cell lymphoma do I have?
• What stage is my disease?
• What are my treatment options?
• Should I consider a clinical trial?

A note from Cleveland Clinic

Peripheral T-cell lymphomas (PTCLs) are a large group of rare blood cancers with a common cause but different genetic makeup. Lymphomas happen when T cells designed to protect your body from intruders turn into cancerous cells. PTCLs affect different areas of your body, which can make them challenging to diagnose and treat. Medical researchers are targeting PTCLs’ genetic changes, finding new ways to treat the conditions. If you have a form of peripheral T-cell lymphoma, ask your healthcare team if there are clinical trials focused on new treatments for your specific condition.