Peripheral T-cell lymphoma

Overview

Peripheral T-cell lymphoma (PTCL) is a fast-growing type of lymphoma. Lymphoma is a cancer that affects the lymphocytes, which are a type of white blood cell. The white blood cells are part of the body's germ-fighting immune system.

There are many types of lymphoma. Lymphoma types are often divided into two categories: Hodgkin lymphoma and non-Hodgkin lymphoma. PTCL is a type of non-Hodgkin lymphoma.

PTCL gets its name from the kind of cell that makes up this cancer. It starts in white blood cells called mature T cells, also known as T lymphocytes. The T cells undergo changes and turn into cancer cells. A cell's DNA holds the instructions that tell the cell what to do and when to die. Because of these DNA changes, the cells stop dying. They usually multiply and form a mass. PTCL typically affects lymph nodes but can spread to any organ, including the liver, spleen, skin, bone marrow and digestive tract, also called the GI tract.

PTCL is not one condition. There are many variations called subtypes that fall under the PTCL umbrella. Similar to other types of lymphoma, PTCL undergoes specific DNA changes that help it hide from the immune system.

Treatment depends on the PTCL subtype and may include chemotherapy, radiation therapy, targeted therapy, immunotherapy, clinical trials and bone marrow transplant, also called bone marrow stem cell transplant.

Causes

Peripheral T-cell lymphoma (PTCL) typically forms when germ-fighting white blood cells get changes in their DNA that turn them into cancer cells. The kind of white blood cells involved in PTCL are mature T cells, also called T lymphocytes. A cell's DNA holds the instructions that tell the cell what to do and when to die. Because of these DNA changes, the cells stop dying. Instead, they multiply and spread. Exactly why these DNA changes happen is not known.

Many types of PTCL usually begin in the lymph nodes and can spread to other organs including the liver, spleen, skin, GI tract and bone marrow.

Some PTCL subtypes also may be affected by a weakened immune system, Celiac disease, and infection with viruses such as Epstein-Barr virus (EBV) and human immunodeficiency virus (HIV).

Complications

Peripheral T-cell lymphoma (PTCL) may cause complications as the cancer grows and spreads to other areas of the body. Complications vary by subtype and may include:

• Swollen lymph nodes: PTCL can cause enlarged lymph nodes that may press on nearby organs.
• Tumor lysis syndrome. This serious condition can happen when cancer cells break down quickly after treatment. When the cells die, they release substances into the bloodstream that can overwhelm the body. This can lead to issues with the kidneys and other organs. Medicines may be given to treat or prevent harmful side effects.

Treatment

Peripheral T-cell lymphoma (PTCL) is not a single condition. There are several subtypes under the PTCL umbrella. Healthcare professionals select treatments based on the PTCL subtype, the stage of the cancer and your overall health.

Since there are many subtypes, the type of first line treatment usually varies. PTCL treatment may include chemotherapy, radiation therapy, immunotherapy, targeted therapy, clinical trials and bone marrow transplant, also called bone marrow stem cell transplant.

Treatments may be more specialized if certain proteins such as CD30 are found on the T lymphocytes.

Chemotherapy

Chemotherapy treats cancer with strong medicines. There are many types of chemotherapy medicines. Most chemotherapy medicines are given through a vein. Some come in pill form.

Radiation therapy

Radiation therapy treats cancer with powerful energy beams. The energy can come from X-rays, protons or other sources. During radiation therapy, you lie on a table while a machine moves around you. The machine directs radiation to precise points in your body.

Targeted therapy

Targeted therapy for cancer is a treatment that uses medicines that attack specific chemicals in the cancer cells. By blocking these chemicals, targeted treatments can cause cancer cells to die.

Immunotherapy

Immunotherapy for cancer is a treatment with medicine that helps the body's immune system fight cancer cells. Typically, the immune system fights off diseases by attacking germs and other cells that shouldn't be in the body. Cancer cells survive by hiding from the immune system. Immunotherapy helps the immune system cells recognize and kill the cancer cells.

Bone marrow transplant

A bone marrow transplant, also called a bone marrow stem cell transplant, involves putting healthy bone marrow stem cells into the body. These cells replace cells hurt by chemotherapy and other treatments.

Autologous stem cell transplant and allogeneic stem cell transplant are procedures that can be used for T-cell lymphoma depending on the type of the disease.

Monitoring after treatment

It is common for peripheral T-cell lymphoma (PTCL) to come back after treatment. This is known as a relapse. The time it takes for a relapse to happen varies by subtype. After treatment, you have follow-up appointments to monitor for relapse. You may need repeat biopsies and imaging to confirm a relapse.

The cancer prognosis tells you how likely it is that the cancer can be treated successfully. Your healthcare team can get a general sense of your outlook based on your peripheral T-cell lymphoma (PTCL) subtype, its stage and other factors such as your overall health.

PTCL is considered fast growing. It can be hard to diagnose and may not be found until it has spread in your body.

Your healthcare team talks with you about treatment options that can give you the best chance for a desirable outcome.

People who are treated for PTCL also might have a higher chance of getting another cancer of a different type after treatment, sometimes many years later. Those cancers may include lung, brain, kidney, bladder, melanoma, Hodgkin lymphoma and acute nonlymphocytic leukemia.

Speak with your healthcare team about your prognosis if you want to know what to expect. Members of your healthcare team can explain what they consider when thinking about your prognosis.

Preparing for an appointment

Make an appointment with a healthcare professional if you have any symptoms that worry you or are not going away.

If your healthcare professional thinks you might have peripheral T-cell lymphoma (PTCL), you may be referred to a doctor who specializes in diseases that affect blood cells, called a hematologist. If a cancer diagnosis is made, you also may be referred to a doctor who specializes in treating cancer, called an oncologist.

It is helpful to be prepared before you go to your appointment. Here is a starting point for getting ready.

What you can do

• Be aware of anything you need to do ahead of time. At the time you make the appointment, be sure to ask whether there's anything you need to do in advance, such as prepare for lab work or other medical tests if needed.
• Write down all the symptoms you have, including any that may not seem related to the reason for the appointment.
• Make a list of all medicines, vitamins and supplements you're taking, how often you take them, and the doses for each.
• Take along a family member or friend if possible. It can be hard to remember all the information you get during an appointment, especially if you feel overwhelmed. Someone who goes with you can take notes and may remember something that you missed or forgot.
• Write down questions to ask your healthcare team.

For peripheral T-cell lymphoma (PTCL), some basic questions to ask include:

• Do I have PTCL or a subtype of PTCL?
• What is the stage of my PTCL?
• Has the PTCL spread to other parts of my body?
• Will I need more tests? If yes, please describe the tests and what they are for.
• What are my treatment options? What is treatment like?
• How much does each treatment prolong my life or increase my chances of a cure?
• What are the potential side effects of each treatment?

Other questions could include:

• How will each treatment affect my daily life?
• Which treatment option do you believe is the best?
• What would you recommend to a friend or family member in my situation?
• How long does it take for the treatment to work?
• What happens if the PTCL comes back after treatment? What are my options then?
• What specialists will make up my care team?
• What will determine follow-up visits?
• Is there any printed information about PTCL you could give me? What websites do you recommend?

Don't hesitate to ask other questions.

What to expect from your doctor

Be prepared to answer questions, such as:

• What are your symptoms?
• When did your symptoms begin?
• Do your symptoms happen all the time or do they come and go?
• How bad are your symptoms?
• What, if anything, seems to make your symptoms better?
• What, if anything, seems to make your symptoms worse?
• What worries you about your diagnosis or treatment?