Cutaneous T-Cell Lymphoma

Treatment and Diagnosis:

Types of Non-Hodgkin Lymphoma

If you have swollen lymph nodes or another symptom that suggests non-Hodgkin lymphoma, your doctor will try to find out what's causing the problem. Your doctor may ask about your personal and family medical history.

You may have some of the following exams and tests:

• Physical exam: Your doctor checks for swollen lymph nodes in your neck, underarms, and groin. Your doctor also checks for a swollen spleen or liver.
• Blood tests: The lab does a complete blood count to check the number of white blood cells. The lab also checks for other cells and substances, such as lactate dehydrogenase (LDH). Lymphoma may cause a high level of LDH.
• Chest x-rays: You may have x-rays to check for swollen lymph nodes or other signs of disease in your chest.
• Biopsy: A biopsy is the only sure way to diagnose lymphoma. Your doctor may remove an entire lymph node (excisional biopsy) or only part of a lymph node (incisional biopsy). A thin needle (fine needle aspiration) usually cannot remove a large enough sample for the pathologist to diagnose lymphoma. Removing an entire lymph node is best. The pathologist uses a microscope to check the tissue for lymphoma cells.

Types of Non-Hodgkin Lymphoma

When lymphoma is found, the pathologist reports the type. There are many types of lymphoma. The most common types are diffuse large B-cell lymphoma and follicular lymphoma.

Lymphomas may be grouped by how quickly they are likely to grow:

• Indolent (also called low-grade) lymphomas grow slowly. They tend to cause few symptoms.
• Aggressive (also called intermediate-grade and high-grade) lymphomas grow and spread more quickly. They tend to cause severe symptoms. Over time, many indolent lymphomas become aggressive lymphomas.

It's a good idea to get a second opinion about the type of lymphoma that you have. The treatment plan varies by the type of lymphoma. A pathologist at a major referral center can review your biopsy.

Staging

Your doctor needs to know the extent (stage) of non-Hodgkin lymphoma to plan the best treatment. Staging is a careful attempt to find out what parts of the body are affected by the disease.

Lymphoma usually starts in a lymph node. It can spread to nearly any other part of the body. For example, it can spread to the liver, lungs, bone, and bone marrow.

Staging may involve one or more of the following tests:

• Bone marrow biopsy: The doctor uses a thick needle to remove a small sample of bone and bone marrow from your hipbone or another large bone. Local anesthesia can help control pain. A pathologist looks for lymphoma cells in the sample.
• CT scan: An x-ray machine linked to a computer takes a series of detailed pictures of your head, neck, chest, abdomen, or pelvis. You may receive an injection of contrast material. Also, you may be asked to drink another type of contrast material. The contrast material makes it easier for the doctor to see swollen lymph nodes and other abnormal areas on the x-ray.
• MRI: Your doctor may order MRI pictures of your spinal cord, bone marrow, or brain. MRI uses a powerful magnet linked to a computer. It makes detailed pictures of tissue on a computer screen or film.
• Ultrasound: An ultrasound device sends out sound waves that you cannot hear. A small hand-held device is held against your body. The waves bounce off nearby tissues, and a computer uses the echoes to create a picture. Tumors may produce echoes that are different from the echoes made by healthy tissues. The picture can show possible tumors.
• Spinal tap: The doctor uses a long, thin needle to remove fluid from the spinal column. Local anesthesia can help control pain. You must lie flat for a few hours afterward so that you don't get a headache. The lab checks the fluid for lymphoma cells or other problems.
• PET scan: You receive an injection of a small amount of radioactive sugar. A machine makes computerized pictures of the sugar being used by cells in your body. Lymphoma cells use sugar faster than normal cells, and areas with lymphoma look brighter on the pictures.

The stage is based on where lymphoma cells are found (in the lymph nodes or in other organs or tissues). The stage also depends on how many areas are affected. The stages of non-Hodgkin lymphoma are as follows:

• Stage I: The lymphoma cells are in one lymph node group (such as in the neck or underarm). Or, if the abnormal cells are not in the lymph nodes, they are in only one part of a tissue or organ (such as the lung, but not the liver or bone marrow).
• Stage II: The lymphoma cells are in at least two lymph node groups on the same side of (either above or below) the diaphragm. Or, the lymphoma cells are in one part of an organ and the lymph nodes near that organ (on the same side of the diaphragm). There may be lymphoma cells in other lymph node groups on the same side of the diaphragm.
Stage III: The lymphoma is in lymph nodes above and below the diaphragm. It also may be found in one part of a tissue or an organ near these lymph node groups.

Stage IV: Lymphoma cells are found in several parts of one or more organs or tissues (in addition to the lymph nodes). Or, it is in the liver, blood, or bone marrow.

Recurrent: The disease returns after treatment.

In addition to these stage numbers, your doctor may also describe the stage as A or B

• A: You have not had weight loss, drenching night sweats, or fevers.
• B: You have had weight loss, drenching night sweats, or fevers

Treatment

• Atchful Waiting
• Chemotherapy
• Biological Therapy
• Radiation Therapy
• Stem Cell Transplantation

Your doctor can describe your treatment choices and the expected results. You and your doctor can work together to develop a treatment plan that meets your needs.

Your doctor may refer you to a specialist, or you may ask for a referral. Specialists who treat non-Hodgkin lymphoma include hematologists, medical oncologists, and radiation oncologists. Your doctor may suggest that you choose an oncologist who specializes in the treatment of lymphoma. Often, such doctors are associated with major academic centers. Your health care team may also include an oncology nurse and a registered dietitian.

The choice of treatment depends mainly on the following:

• The type of non-Hodgkin lymphoma (for example, follicular lymphoma)
• Its stage (where the lymphoma is found)
• How quickly the cancer is growing (whether it is indolent or aggressive lymphoma)
• Your age
• Whether you have other health problems

If you have indolent non-Hodgkin lymphoma without symptoms, you may not need treatment for the cancer right away. The doctor watches your health closely so that treatment can start when you begin to have symptoms. Not getting cancer treatment right away is called watchful waiting.

If you have indolent lymphoma with symptoms, you will probably receive chemotherapy and biological therapy. Radiation therapy may be used for people with Stage I or Stage II lymphoma.

If you have aggressive lymphoma, the treatment is usually chemotherapy and biological therapy. Radiation therapy also may be used.

If non-Hodgkin lymphoma comes back after treatment, doctors call this a relapse or recurrence. People with lymphoma that comes back after treatment may receive high doses of chemotherapy, radiation therapy, or both, followed by stem cell transplantation.

You may want to know about side effects and how treatment may change your normal activities. Because chemotherapy and radiation therapy often damage healthy cells and tissues, side effects are common. Side effects may not be the same for each person, and they may change from one treatment session to the next. Before treatment starts, your health care team will explain possible side effects and suggest ways to help you manage them.

At any stage of the disease, you can have supportive care. Supportive care is treatment to control pain and other symptoms, to relieve the side effects of therapy, and to help you cope with the feelings that a diagnosis of cancer can bring.

You may want to talk to your doctor about taking part in a clinical trial, a research study of new treatment methods.

Watchful Waiting

People who choose watchful waiting put off having cancer treatment until they have symptoms. Doctors sometimes suggest watchful waiting for people with indolent lymphoma. People with indolent lymphoma may not have problems that require cancer treatment for a long time. Sometimes the tumor may even shrink for a while without therapy. By putting off treatment, they can avoid the side effects of chemotherapy or radiation therapy.

If you and your doctor agree that watchful waiting is a good idea, the doctor will check you regularly (every 3 months). You will receive treatment if symptoms occur or get worse.

Some people do not choose watchful waiting because they don't want to worry about having cancer that is not treated. Those who choose watchful waiting but later become worried should discuss their feelings with the doctor.

Chemotherapy

Chemotherapy for lymphoma uses drugs to kill lymphoma cells. It is called systemic therapy because the drugs travel through the bloodstream. The drugs can reach lymphoma cells in almost all parts of the body.

You may receive chemotherapy by mouth, through a vein, or in the space around the spinal cord. Treatment is usually in an outpatient part of the hospital, at the doctor's office, or at home. Some people need to stay in the hospital during treatment.

Chemotherapy is given in cycles. You have a treatment period followed by a rest period. The length of the rest period and the number of treatment cycles depend on the stage of your disease and on the anticancer drugs used.

If you have lymphoma in the stomach caused by H. pylori infection, your doctor may treat this lymphoma with antibiotics. After the drug cures the infection, the lymphoma also may go away.

The side effects depend mainly on which drugs are given and how much. The drugs can harm normal cells that divide rapidly:

• Blood cells: When chemotherapy lowers your levels of healthy blood cells, you are more likely to get infections, bruise or bleed easily, and feel very weak and tired. Your health care team gives you blood tests to check for low levels of blood cells. If levels are low, there are medicines that can help your body make new blood cells.
• Cells in hair roots: Chemotherapy may cause hair loss. If you lose your hair, it will grow back, but it may be somewhat different in color and texture.
• Cells that line the digestive tract: Chemotherapy can cause poor appetite, nausea and vomiting, diarrhea, trouble swallowing, or mouth and lip sores. Ask your health care team about medicines or other treatments that help with these problems.

The drugs used for non-Hodgkin lymphoma also may cause skin rashes or blisters, and headaches or other aches. Your skin may become darker. Your nails may develop ridges or dark bands.

Biological Therapy

People with certain types of non-Hodgkin lymphoma may have biological therapy. This type of treatment helps the immune system fight cancer.

Monoclonal antibodies are the type of biological therapy used for lymphoma. They are proteins made in the lab that can bind to cancer cells. They help the immune system kill lymphoma cells. People receive this treatment through a vein at the doctor's office, clinic, or hospital.

Flu-like symptoms such as fever, chills, headache, weakness, and nausea may occur. Most side effects are easy to treat. Rarely, a person may have more serious side effects, such as breathing problems, low blood pressure, or severe skin rashes. Your doctor or nurse can tell you about the side effects that you can expect and how to manage them.

Radiation Therapy

Radiation therapy (also called radiotherapy) uses high-energy rays to kill lymphoma cells. It can shrink tumors and help control pain.

Two types of radiation therapy are used for people with lymphoma:

• External radiation: A large machine aims the rays at the part of the body where lymphoma cells have collected. This is local therapy because it affects cells in the treated area only. Most people go to a hospital or clinic for treatment 5 days a week for several weeks.
• Systemic radiation: Some people with lymphoma receive an injection of radioactive material that travels throughout the body. The radioactive material is bound to monoclonal antibodies that seek out lymphoma cells. The radiation destroys the lymphoma cells.

The side effects of radiation therapy depend mainly on the type of radiation therapy, the dose of radiation, and the part of the body that is treated. For example, external radiation to your abdomen can cause nausea, vomiting, and diarrhea. When your chest and neck are treated, you may have a dry, sore throat and some trouble swallowing. In addition, your skin in the treated area may become red, dry, and tender. You also may lose your hair in the treated area.

You are likely to become very tired during external radiation therapy, especially in the later weeks of treatment. Resting is important, but doctors usually advise people to try to stay as active as they can.

People who get systemic radiation also may feel very tired. They may be more likely to get infections.

If you have radiation therapy and chemotherapy at the same time, your side effects may be worse. The side effects can be distressing. You can talk with your doctor about ways to relieve them.

Stem Cell Transplantation

If lymphoma returns after treatment, you may receive stem cell transplantation. A transplant of your own blood-forming stem cells allows you to receive high doses of chemotherapy, radiation therapy, or both. The high doses destroy both lymphoma cells and healthy blood cells in the bone marrow.

Stem cell transplants take place in the hospital. After you receive high-dose treatment, healthy blood-forming stem cells are given to you through a flexible tube placed in a large vein in your neck or chest area. New blood cells develop from the transplanted stem cells.

The stem cells may come from your own body or from a donor: :

• Autologous stem cell transplantation: This type of transplant uses your own stem cells. Your stem cells are removed before high-dose treatment. The cells may be treated to kill lymphoma cells that may be present. The stem cells are frozen and stored. After you receive high-dose treatment, the stored stem cells are thawed and returned to you.
• Allogeneic stem cell transplantation: Sometimes healthy stem cells from a donor are available. Your brother, sister, or parent may be the donor. Or the stem cells may come from an unrelated donor. Doctors use blood tests to be sure the donor's cells match your cells.
• Syngeneic stem cell transplantation: This type of transplant uses stem cells from a patient's healthy identical twin.

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