T-cell lymphoma

Overview

T-cell lymphomas are a type of lymphoma. Lymphoma is cancer that affects the lymphatic system. The lymphatic system is made up of organs, glands, tubelike vessels and clusters of cells called lymph nodes. It's part of the body's germ-fighting immune system. T-cell lymphomas can affect lymph nodes, blood, bone marrow, and other tissues throughout the body, such as in the gastrointestinal system, liver, nasal cavity and skin.

There are many types of lymphoma. Lymphoma types are often divided into two categories: Hodgkin lymphoma and non-Hodgkin lymphoma. T-cell lymphomas are a type of non-Hodgkin lymphoma. There are many subtypes of T-cell lymphomas.

T-cell lymphomas get their name from the kind of cell that makes up these cancers. These cancers start in germ-fighting white blood cells called T cells. T cells also are called T lymphocytes.

Treatment for T-cell lymphomas may include a "watch and wait" approach, chemotherapy, immunotherapy, radiation therapy, targeted therapy, clinical trials, and bone marrow transplant, also called bone marrow stem cell transplant.

Types

There are many types of T-cell lymphomas, including:

• Adult T-cell leukemia/lymphoma. This type is caused by a long-term infection with human T-lymphotropic virus 1 (HTLV-1). It is mostly seen in parts of Japan, the Caribbean and Central Africa.
• Anaplastic large cell lymphoma (ALCL). This type has two forms: ALK-positive and ALK-negative forms. ALK-positive disease is more common in younger people and usually responds better to therapy. ALCL also can be linked to breast implants. This is known as breast implant-associated anaplastic large cell lymphoma.
• Cutaneous T-cell lymphomas (CTCL). This type affects the skin and is not found in the lymph nodes or elsewhere in the body. Two common forms of CTCL are mycosis fungoides and Sézary syndrome. CTCL usually shows up first as itchy patches or plaques on the skin.
• Extranodal NK/T-cell lymphoma. This type is linked to Epstein-Barr virus (EBV) infection. It often begins in the nasal passages and can cause facial swelling or nosebleeds.
• Hepatosplenic T-cell lymphoma. This type affects the spleen, liver and bone marrow. It is more common in people who have been immunocompromised.
• Intestinal T-cell lymphoma. This type can affect the intestines, lymph nodes, liver, spleen, lungs and bone marrow. The most common form is enteropathy-associated T-cell lymphoma. It can develop in people with celiac disease. Symptoms of intestinal T-cell lymphoma include belly pain and discomfort, fatigue, nausea, vomiting and diarrhea.
• Nodal T-follicular helper cell lymphoma, angioimmunoblastic-type. This type starts in a type of cell called T follicular helper cells. It mostly affects the lymph nodes. People with nodal T-follicular helper cell lymphoma also often have Epstein-Barr virus (EBV) infection. This type used to be called angioimmunoblastic T-cell lymphoma.
• Peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS). This is a category for T-cell lymphomas that do not fit other groups. PTCL-NOS typically involves the lymph nodes, liver, bone marrow, gastrointestinal tract and skin.

Healthcare professionals may describe T-cell lymphomas as either aggressive or indolent. Aggressive lymphomas grow quickly and may have more symptoms. Indolent lymphomas grow slowly and may not have symptoms at first. Treatments may vary depending on if the lymphoma is aggressive or indolent. Some indolent lymphomas can transform into aggressive lymphomas.

Causes

It's not clear what causes T-cell lymphomas. Cancer happens when cells develop changes in their DNA. A cell's DNA holds the instructions that tell the cell what to do. In healthy cells, the DNA gives instructions to grow and multiply at a set rate. The instructions tell the cells to die at a set time. In cancer cells, the DNA changes give different instructions. The changes tell the cancer cells to make many more cells quickly. Cancer cells can keep living when healthy cells would die. This causes too many cells.

T-cell lymphomas get their names from the kind of cell that makes up these cancers. These cancers start in germ-fighting white blood cells called T cells. T cells also are called T lymphocytes.

Complications

A complication of T-cell lymphomas is the risk of the cancer coming back after treatment, called a relapse. Sometimes the relapse happens years later. Some people may have multiple relapses. People who relapse have a worse prognosis.

Treatment

Treatments for T-cell lymphomas may include a "watch and wait" approach, chemotherapy, immunotherapy, radiation therapy, targeted therapy, clinical trials, and bone marrow transplant, also called bone marrow stem cell transplant. Which treatment is best for you depends on the type of T-cell lymphoma you have and the extent of your cancer, called the stage. Your healthcare team also considers how quickly the cancer is growing, your overall health and what you prefer.

Watch and wait

If your T-cell lymphoma doesn't cause symptoms, you might not need treatment right away. Instead, you may have checkups every few months. The checkups help your healthcare team watch your condition to see if your cancer is growing.

Chemotherapy

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

Chemotherapy may be used to treat some T-cell lymphomas. You may be given several chemotherapy medicines at one time. Chemotherapy may be combined with immunotherapy or other medicines. Chemotherapy also may be an option when other treatments haven't worked, known as refractory T-cell lymphoma, or for cancer that comes back after treatment, called relapsed T-cell lymphoma.

Immunotherapy

Immunotherapy for cancer is a treatment with medicine that helps the body's immune system kill cancer cells. 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 find and kill the cancer cells.

Immunotherapy may be used to treat some T-cell lymphomas. It may be combined with chemotherapy medicines. Immunotherapy also may be an option for relapsed or refractory T-cell lymphoma.

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.

Radiation therapy may be used to treat some T-cell lymphomas. It may be combined with chemotherapy or other medicines. Radiation therapy also may be used to ease symptoms and improve quality of life for people with some types of T-cell lymphomas.

Targeted therapy

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

Targeted therapy may be used to treat some T-cell lymphomas. It also may be an option for some refractory or relapsed T-cell lymphomas.

Clinical trials

Clinical trials are studies of new treatments. These studies provide a chance to try the latest treatments. The risk of side effects might not be known. Ask your healthcare team if you might be able to be in a clinical trial.

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. Stem cells can come from your own body, called an autologous transplant. Stem cells also can come from a donor, called an allogeneic transplant.

A bone marrow transplant may only be an option for some types of T-cell lymphoma and for people who are younger and in good health. Chemotherapy is typically done before the transplant to suppress the immune system and bone marrow.

Treatments for cutaneous T-cell lymphomas

Treatments for cutaneous T-cell lymphomas may include topical medicines and phototherapy. Topical medicines are placed directly on the patches or plaques on the skin. They may include corticosteroids, retinoids and topical chemotherapy. Phototherapy uses lights combined with medicines to treat some cancers of the skin.

Monitoring after treatment

After treatment is complete, you may have frequent follow-up appointments to see if the cancer has come back, known as a relapse. You may have repeat blood and imaging tests and, if needed, biopsies to check for relapse.

Side effects of treatment

Treatments for T-cell lymphomas may have side effects. These may include nausea, vomiting, fatigue, fever, rash, diarrhea and more. Harmful side effects from treatments may include:

• Bone marrow suppression. When the bone marrow is suppressed, it can't produce enough blood cells, including white blood cells. White blood cells help fight infections. So with fewer white blood cells, you have a higher risk of infections.
• Febrile neutropenia. Febrile neutropenia is a serious condition that can happen in people being treated for cancer. Febrile means having a fever. Neutropenia means having a low number of neutrophils, which are a type of white blood cell that helps fight infections. In febrile neutropenia, the body is trying to fight an infection but doesn't have enough neutrophils.
• Medicine toxicity. Some medicines used to treat cancer can be harmful to the body. They can cause organ damage and other issues. Whether a medicine is toxic depends on the type and how much you take. There are many medicine options for T-cell lymphoma. Your healthcare team can work with you to find the best medicines for you.
• Reactivating viruses. If you have had a viral infection in the past, such as hepatitis B or hepatitis C, some treatments can cause the virus to become active again. This can lead to liver inflammation, liver damage and other complications.
• Tumor lysis syndrome. Tumor lysis syndrome is a serious condition that can happen when cancer cells break down quickly after treatment. When these 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.

Preparing for an appointment

Make an appointment with a healthcare professional if you have any symptoms that worry you.

If your healthcare professional thinks you might have a T-cell lymphoma, 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.

Because appointments can be brief, it's a good idea to be prepared. Here's some information to help you get ready.

What you can do

• Be aware of anything you need to do ahead of time. At the time you make the appointment, ask if there's anything you need to do in advance, such as restrict your diet.
• Write down symptoms you have, including any that may not seem related to the reason for which you scheduled the appointment.
• Write down important personal information, including major stresses or recent life changes.
• Make a list of all medicines, vitamins and supplements you're taking and the doses.
• Take a family member or friend along. It can be hard to remember all the information you get during an appointment. Someone who goes with you may remember something that you missed or forgot.
• Write down questions to ask your healthcare team.

For T-cell lymphomas, some basic questions to ask include:

• Do I have a T-cell lymphoma?
• What type of T-cell lymphoma do I have?
• What is the stage of my T-cell lymphoma?
• Has my T-cell lymphoma spread to other parts of my body?
• Will I need more tests?
• What are the treatment options?
• How much does each treatment prolong my life or increase my chances of a cure?
• What are the potential side effects of each treatment?
• How will each treatment affect my daily life?
• Is there one treatment option you believe is the best?
• What would you recommend to a friend or family member in my situation?
• Should I see a specialist?
• Are there any brochures or other printed material that I can take with me? What websites do you recommend?
• What will determine whether I should plan for a follow-up visit?

Don't hesitate to ask other questions.

What to expect from your doctor

Be prepared to answer questions, such as:

• When did your symptoms begin?
• Do your symptoms happen all the time or do you have them now and then?
• How bad are your symptoms?
• What, if anything, seems to make symptoms better?
• What, if anything, seems to worsen your symptoms?
• Have you had any fevers, night sweats or weight loss?
• Have you noticed any lumps, swelling or pain anywhere in your body?
• Have you had any chest pain, coughing or trouble breathing?
• Have you had any recent infections?
• Do you have any other health conditions, such as diabetes, heart disease or kidney disease?
• What are your biggest worries about your diagnosis or treatment?