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Adult Non-Hodgkinfs Lymphoma
Lymphoma

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General Information About Adult Non-Hodgkin’s Lymphoma

Adult non-Hodgkinfs lymphoma is a disease in which malignant (cancer) cells form in the lymph system.

The lymph system is part of the immune system and is made up of the following:

  • Lymph: Colorless, watery fluid that travels through the lymph system and carries white blood cells called lymphocytes. Lymphocytes protect the body against infections and the growth of tumors.

  • Lymph vessels: A network of thin tubes that collect lymph from different parts of the body and return it to the bloodstream.

  • Lymph nodes: Small, bean-shaped structures that filter substances in lymph and help fight infection and disease. Lymph nodes are located along the network of lymph vessels found throughout the body. Clusters of lymph nodes are found in the underarm, pelvis, neck, abdomen, and groin.

  • Spleen: An organ that produces lymphocytes, filters the blood, stores blood cells, and destroys old blood cells. It is located on the left side of the abdomen near the stomach.

  • Thymus: An organ in which lymphocytes grow and multiply. The thymus is in the chest behind the breastbone.

  • Tonsils: Two small masses of lymph tissue at the back of the throat. The tonsils produce lymphocytes.

  • Bone marrow: The soft, spongy tissue in the center of large bones. Bone marrow produces white blood cells, red blood cells, and platelets.

Because lymph tissue is found throughout the body, adult non-Hodgkinfs lymphoma can begin in almost any part of the body. Cancer can spread to the liver and many other organs and tissues.

Non-Hodgkinfs lymphoma can occur in both adults and children. Treatment for children, however, is different than treatment for adults.

There are many different types of lymphoma.

Lymphomas are divided into two general types: Hodgkinfs lymphoma and non-Hodgkinfs lymphoma. This summary refers to the treatment of adult non-Hodgkinfs lymphoma.

Age, gender, and a weakened immune system can affect the risk of developing adult non-Hodgkinfs lymphoma.

Risk factors for adult non-Hodgkinfs lymphoma include the following:

  • Being older, male, or white.

  • Having one of the following medical conditions:
    • An inherited immune disorder.
    • An autoimmune disease.
    • HIV/AIDS.
    • Human T-lymphotrophic virus type I or Epstein-Barr virus.
    • A history of Helicobacter pylori infection.

  • Taking immunosuppressant drugs after an organ transplant.

  • Being exposed to certain pesticides.

  • A diet high in meats and fat.

  • Past treatment for Hodgkinfs lymphoma or with radiation.

Possible signs of adult non-Hodgkinfs lymphoma include fever, sweating, fatigue, and weight loss.

These and other symptoms may be caused by adult non-Hodgkinfs lymphoma or by other conditions. A doctor should be consulted if any of the following problems occur:

  • Painless swelling in the lymph nodes in the neck, underarm, groin, or stomach.
  • Fever (unexplained).
  • Drenching night sweats.
  • Constant tiredness.
  • Weight loss (unexplained) in the past 6 months.
  • Skin rash or itchy skin.
  • Pain in the chest, abdomen, or bones (unexplained).

Tests that examine the body and lymph system are used to help detect (find) and diagnose adult non-Hodgkinfs lymphoma.

The following tests and procedures may be used:

  • Physical exam and history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patientfs health habits and past illnesses and treatments will also be taken.

  • Complete blood count: A procedure in which a sample of blood is drawn and checked for the following:
    • The number of red blood cells, white blood cells, and platelets.
    • The amount of hemoglobin (the protein that carries oxygen) in the red blood cells.
    • The portion of the sample made up of red blood cells.

  • Blood chemistry studies: A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that produces it.

  • Lymph node biopsy: The removal of all or part of a lymph node. A pathologist views the tissue under a microscope to look for cancer cells. One of the following types of biopsies may be done:
    • Excisional biopsy: The removal of an entire lymph node.
    • Incisional biopsy: The removal of part of a lymph node.
    • Core biopsy: The removal of part of a lymph node using a wide needle.
    • Needle biopsy: The removal of part of a lymph node using a thin needle. This procedure is also called a fine-needle aspiration biopsy.

  • Bone marrow biopsy: The removal of a small piece of bone and bone marrow by inserting a needle into the hipbone or breastbone. A pathologist views both the bone and bone marrow samples under a microscope to look for signs of cancer.

Certain factors affect prognosis (chance of recovery) and treatment options.

The prognosis (chance of recovery) and treatment options depend on the following:

  • The stage of the cancer.
  • The type of non-Hodgkin's lymphoma.
  • The patientfs age and general health.
  • Whether the lymphoma has just been diagnosed or has recurred (come back).

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Stages of Adult Non-Hodgkinfs Lymphoma

After adult non-Hodgkinfs lymphoma has been diagnosed, tests are done to find out if cancer cells have spread within the lymph system or to other parts of the body.

The process used to find out the type of cancer and if cancer cells have spread within the lymph system or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage of the disease in order to plan treatment. The following tests and procedures may be used in the staging process:

  • Chest x-ray: An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body.

  • CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.

  • PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radionuclide glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells.

  • MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI).

  • Gallium scan: A procedure to detect areas of the body where cells, such as cancer cells, are dividing rapidly. A very small amount of radioactive material, gallium, is injected into a vein and travels through the bloodstream. The gallium collects in the bones or other tissues (organs) and is detected by a scanner.

  • Bone marrow biopsy: The removal of a small piece of bone and bone marrow by inserting a needle into the hipbone or breastbone. A pathologist views both the bone and bone marrow samples under a microscope to look for signs of cancer.

  • Lumbar puncture: A procedure used to collect cerebrospinal fluid from the spinal column. This is done by placing a needle into the spinal column. This procedure is also called an LP or spinal tap.

The following stages are used for adult non-Hodgkinfs lymphoma:

Stage I

Stage I adult non-Hodgkinfs lymphoma is divided into stage I and stage IE (gEh stands for extranodal and means that the cancer is found in an organ or tissue other than the lymph nodes).

  • Stage I: Cancer is found in a single lymph node area.

  • Stage IE: Cancer is found in an organ or tissue other than the lymph nodes.

Stage II

Stage II adult non-Hodgkinfs lymphoma is divided into stage II and stage IIE (gEh stands for extranodal and means that the cancer is found in an organ or tissue other than the lymph nodes).

  • Stage II: Cancer is found in two or more lymph node areas on the same side of the diaphragm (the thin muscle below the lungs that helps breathing and separates the chest from the abdomen).

  • Stage IIE: Cancer is found in an organ or tissue other than the lymph nodes and may have spread to one or more lymph nodes on the same side of the diaphragm.

Stage III

Stage III adult non-Hodgkinfs lymphoma is divided into stage III, stage IIIE (gEh stands for extranodal and means that the cancer is found in an organ or tissue other than the lymph nodes), stage IIIS (gSh stands for spleen and means that the cancer is found in the spleen), and stage IIIS+E.

  • Stage III: Cancer is found in lymph node areas on both sides of the diaphragm.

  • Stage IIIE: Cancer is found in lymph node areas on both sides of the diaphragm and in one area of a nearby organ or tissue other than the lymph nodes.

  • Stage IIIS: Cancer is found in lymph node areas on both sides of the diaphragm and in the spleen.

  • Stage IIIS+E: Cancer is found in lymph node areas on both sides of the diaphragm, in one area of a nearby organ or tissue, and in the spleen.

Stage IV

In stage IV adult non-Hodgkinfs lymphoma, the cancer either:

  • is found throughout at least one organ or tissue other than the lymph nodes and may be in lymph nodes near this organ or tissue; or

  • has spread throughout one organ or tissue other than the lymph nodes and has spread to lymph nodes far away from that organ.

Adult non-Hodgkinfs lymphomas are also described in terms of how fast they grow and the location of affected lymph nodes.

Indolent or aggressive:

  • Indolent lymphomas: These tend to grow and spread slowly and have few symptoms. They are also called low-grade lymphomas.

  • Aggressive lymphomas: These grow and spread quickly and have severe symptoms. Lymphoblastic lymphoma, diffuse small noncleaved cell lymphoma and Burkitt lymphoma are 3 types of aggressive adult non-Hodgkinfs lymphoma. Aggressive lymphomas are seen more frequently in patients who are HIV-positive (AIDS-related lymphoma). Aggressive lymphomas are also called intermediate-grade and high-grade lymphomas.

Contiguous or noncontiguous:

  • Contiguous lymphomas: Lymphomas in which the lymph nodes containing cancer are next to each other.

  • Noncontiguous lymphomas: Lymphomas in which the lymph nodes containing cancer are not next to each other, but are on the same side of the diaphragm.

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Recurrent Adult Non-Hodgkinfs Lymphoma

Recurrent adult non-Hodgkinfs lymphoma is cancer that has recurred (come back) after it has been treated. The lymphoma may come back in the lymph system or in other parts of the body. Indolent lymphoma may come back as aggressive lymphoma. Aggressive lymphoma may come back as indolent lymphoma.

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Treatment Option Overview

There are different types of treatment for patients with non-Hodgkinfs lymphoma.

Different types of treatment are available for patients with non-Hodgkinfs lymphoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. Before starting treatment, patients may want to think about taking part in a clinical trial. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment.

Clinical trials are taking place in many parts of the country. Choosing the most appropriate cancer treatment is a decision that ideally involves the patient, family, and health care team.

Three types of standard treatment are used:

Radiation therapy

Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells. There are two types of radiation therapy. External radiation therapy uses a machine outside the body to send radiation toward the cancer. Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated.

Chemotherapy

Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping the cells from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the spinal column, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). To treat certain types of adult non-Hodgkinfs lymphoma that spread to the brain, CNS prophylaxis (chemotherapy given to kill cancer cells in the brain or spinal cord) may be used. The way the chemotherapy is given depends on the type and stage of the cancer being treated.

Watchful waiting

Watchful waiting is closely monitoring a patientfs condition without giving any treatment until symptoms appear or change.

Other types of treatment are being tested in clinical trials.

Biologic therapy

Biologic therapy is a treatment that uses the patientfs immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the bodyfs natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy.

There are different types of biologic therapy used in treating adult non-Hodgkinfs lymphoma, including the following:

  • Monoclonal antibody therapy: A cancer treatment that uses antibodies made in the laboratory, from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells or block their growth. Monoclonal antibodies are given by infusion. They may be used alone or to deliver drugs, toxins, or radioactive material directly to cancer cells.

  • Vaccine therapy: Vaccine therapy uses a substance or group of substances meant to cause the immune system (the complex group of organs and cells that defends the body against infection or disease) to respond to a tumor and kill it.

High-dose chemotherapy with stem cell transplantation

High-dose chemotherapy with stem cell transplantation is a method of giving high doses of chemotherapy and replacing blood-forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the bodyfs blood cells.

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Treatment Options by Stage

Indolent, Stage I and Contiguous Stage II Adult Non-Hodgkinfs Lymphoma

Treatment of indolent, stage I and contiguous stage II adult non-Hodgkinfs lymphoma may include the following:

  • Radiation therapy directed at the area where cancer is located.

  • Radiation therapy directed at the area where cancer is located and nearby lymph nodes.

  • Chemotherapy with radiation therapy.

  • Chemotherapy alone or watchful waiting for patients who cannot have radiation therapy.

  • Radiation therapy directed at part or all of the lymph system.

Aggressive, Stage I and Contiguous Stage II Adult Non-Hodgkinfs Lymphoma

Treatment of aggressive, stage I and contiguous stage II adult non-Hodgkinfs lymphoma is usually combination chemotherapy (chemotherapy using more than one drug) with radiation therapy. Chemotherapy alone may also be used.

Indolent, Noncontiguous Stage II/III/IV Adult Non-Hodgkinfs Lymphoma

Treatment of indolent, noncontiguous stage II/III/IV adult non-Hodgkinfs lymphoma may include the following:

  • Watchful waiting for patients who do not have symptoms.

  • Chemotherapy with or without steroids (drugs used to relieve swelling and inflammation).

  • Combination chemotherapy with steroids.

  • Monoclonal antibody therapy with or without combination chemotherapy.

  • Radiolabeled monoclonal antibody therapy.

  • Radiation therapy directed at the area where cancer is located and nearby lymph nodes, for patients who have stage III disease.

  • A clinical trial of chemotherapy and total-body irradiation (radiation therapy to the entire body) followed by autologous or allogeneic stem cell transplantation.

  • A clinical trial of chemotherapy with or without vaccine therapy.

Aggressive, Noncontiguous Stage II/III/IV Adult Non-Hodgkinfs Lymphoma

Treatment of aggressive, noncontiguous stage II/III/IV adult non-Hodgkinfs lymphoma may include the following:

  • Combination chemotherapy alone.

  • Combination chemotherapy with radiation therapy or monoclonal antibody therapy.

  • Combination chemotherapy with CNS prophylaxis.

  • A clinical trial of autologous or allogeneic stem cell transplantation for patients who are likely to relapse.

Adult Lymphoblastic Lymphoma

Treatment of adult lymphoblastic lymphoma may include the following:

  • Combination chemotherapy and CNS prophylaxis.

  • A clinical trial of autologous or allogeneic stem cell transplantation.

Diffuse Small Noncleaved Cell/Burkitt Lymphoma

Treatment of adult diffuse small noncleaved cell/Burkitt lymphoma may include the following:

  • Combination chemotherapy and CNS prophylaxis.

  • A clinical trial of combination chemotherapy.

  • A clinical trial of autologous or allogeneic stem cell transplantation.

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Treatment Options for Recurrent Adult Non-Hodgkinfs Lymphoma

Indolent, Recurrent Adult Non-Hodgkinfs Lymphoma

Treatment of indolent, recurrent adult non-Hodgkinfs lymphoma may include the following:

  • Chemotherapy with one or more drugs.

  • Radiation therapy.

  • Radiation therapy and/or chemotherapy as palliative therapy to relieve symptoms and improve quality of life.

  • Monoclonal antibody therapy.

  • A clinical trial of radiolabeled monoclonal antibody therapy.

  • A clinical trial of monoclonal antibody therapy as palliative therapy to relieve symptoms and improve quality of life.

  • A clinical trial of autologous or allogeneic stem cell transplantation.

Treatment of indolent lymphoma that comes back as aggressive lymphoma may include the following:

  • A clinical trial of autologous or allogeneic stem cell transplantation.

  • A clinical trial of combination chemotherapy followed by radiation therapy or stem cell transplantation and radiation therapy.

  • A clinical trial of stem cell transplantation.

  • A clinical trial of monoclonal antibody therapy.

  • A clinical trial of radiolabeled monoclonal antibody therapy.

  • A clinical trial of continuous-infusion chemotherapy (chemotherapy administered into a blood vessel over a long period of time).

Aggressive, Recurrent Adult Non-Hodgkinfs Lymphoma

Treatment of aggressive, recurrent adult non-Hodgkinfs lymphoma may include the following:

  • Stem cell transplantation.

  • Monoclonal antibody therapy.

  • A clinical trial of autologous or allogeneic stem cell transplantation.

  • A clinical trial of combination chemotherapy followed by radiation therapy or stem cell transplantation and radiation therapy.

  • A clinical trial of radiolabeled monoclonal antibody therapy.

  • A clinical trial of continuous-infusion chemotherapy.

Treatment of aggressive lymphoma that comes back as indolent lymphoma may include the following:

  • Chemotherapy.
  • Palliative therapy to relieve symptoms and improve quality of life.

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Lymphoma