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Definition: Lymphoma from Rehabilitation Medicine Quick Reference: Spine
Description

Lymphoma is a malignant disease of lymphoreticular origin that usually arises from the lymph nodes.

Etiology/Types
  • Hodgkin’s lymphoma

  • Non-Hodgkin’s lymphoma

    • B cell lymphoma (most common)

    • T cell lymphoma

    • Natural killer (NK) cell lymphoma

    • Immunodeficiency-associated lymphoproliferative disorders

  • Staging (stages I to IV) and grading from low to high for non-Hodgkin’s lymphoma allow for further classification

  • Unknown etiology

Epidemiology
  • The annual incidence is 40 to 60 cases per million individuals.

  • Bony involvement is due to hematogenous spread or direct extension by the tumor.

  • Most commonly occurs between 20 and 60 years of age.

  • Male to female ratio of 2:1

  • The lumbosacral spine is involved in 55% of cases, the thoracic spine 34% of cases, and the cervical spine 11% of cases.

Pathogenesis
  • Generally unknown

  • Extraosseous lesions may be related to osteoclastic cytokines produced by the malignant cells.

Risk Factors
  • Autoimmune disease

  • Epstein-Barr virus infection

  • HIV or HTLV-1 infection

  • Increasing age

  • Positive family history

Clinical Features
  • Persistent pain over the affected bony region

  • Most often invades the axial spine

Natural History
  • Remission is possible in certain types of lymphomas if the disease is not too extensive.

  • 5-year survival rate approaches 50%.

Diagnosis
Differential diagnosis
  • Eosinophilic granuloma

  • Neoplasm of the breast or prostate

  • Paget’s disease

History
  • Persistent pain over the affected bony area

  • Pain is worsened with recumbent position.

  • Increased bony pain with the consumption of alcohol

  • Multiple lesions may result in constitutional symptoms such as fever.

  • Neurologic manifestations with invasion of the peripheral nerves or the central spinal canal

Exam
  • Bony tenderness to palpation and a soft tissue mass of the affected bone

  • Characteristic tenderness over a pathologic compression fracture

  • Neurologic deficits

  • Lymphadenopathy and splenomegaly in patients with generalized disease

Testing
  • Laboratory studies are usually normal, although anemia with an increased erythrocyte sedimentation rate and increased serum proteins may indicate extension into other tissues.

  • Histologic findings include Reed-Steinberg cells, atypical mononuclear cells

  • X-rays may demonstrate lytic, sclerotic, periosteal lesions, or a compression fracture

  • Primarily invades the vertebral body followed by the posterior elements.

  • Bone scan may be used to detect multiple lesions and for monitoring the response to chemotherapy.

  • CT is used for staging purposes and to assess bony involvement.

  • MRI may detect early changes in bone and lymph node involvement.

  • Positron emission tomography (PET) scan

  • Bone marrow aspiration

Pitfalls
  • Inadequate staging

Red Flags
  • Acute onset of paraparesis or cauda equina syndron me with epidural lymphomas

Treatment
Medical
  • Radiation therapy and/or chemotherapy based on staging

  • Rituximab (anti-CD-20 monoclonal antibodies)

  • Stem cell transplant

Exercises
  • None

Modalities
  • None

Injection
  • None

Surgical
  • Surgical decompression is considered in younger patients with rapidly progressive paralysis.

Consults
  • Hematology oncology

  • Radiation oncology

  • Neurologic or orthopedic-spine surgery

Complications of treatment
  • Complications related to chemotherapeutic agents, surgery, and radiation

Prognosis
  • Significant deficits in mobility may be possible with epidural disease

  • The 5-year survival rate is 50%.

Helpful Hints
  • Plain radiographs are an easy tool to use to evaluate for the localized bony manifestations of lymphoma.

Suggested Readings
  • Eichler, A F; Batchelor, T T. Primary central nervous system lymphoma: presentation, diagnosis and staging. Neurosurg Focus. 2006; 21 (5):E15.
  • Citow, J S; Rini, B; Wollmann, R; Macdonald, R L. Isolated, primary extranodal Hodgkin’s disease of the spine: case report. Neurosurgery. 2001; 49 (2): 453-456.

Summary Article: Lymphoma from Health Reference Series: Cancer Sourcebook
Primary CNS Lymphoma

Text in this section is excerpted from “Primary CNS Lymphoma Treatment,” National Cancer Institute at the National Institutes of Health (NIH), May 28, 2015.

General Information About Primary CNS Lymphoma
Primary central nervous system (CNS) lymphoma is a disease in which malignant (cancer) cells form in the lymph tissue of the brain and / or spinal cord.

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 lymph, lymph vessels, lymph nodes, spleen, thymus, tonsils, and bone marrow. Lymphocytes (carried in the lymph) travel in and out of the central nervous system (CNS). It is thought that some of these lymphocytes become malignant and cause lymphoma to form in the CNS. Primary CNS lymphoma can start in the brain, spinal cord, or meninges (the layers that form the outer covering of the brain). Because the eye is so close to the brain, primary CNS lymphoma can also start in the eye (called ocular lymphoma).

Having a weakened immune system may increase the risk of developing primary CNS lymphoma.

Anything that increases your chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your doctor if you think you may be at risk.

Primary CNS lymphoma may occur in patients who have acquired immunodeficiency syndrome (AIDS) or other disorders of the immune system or who have had a kidney transplant.

Tests that examine the eyes, brain, and spinal cord are used to detect (find) and diagnose primary CNS 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's health habits and past illnesses and treatments will also be taken.

  • Neurological exam: A series of questions and tests to check the brain, spinal cord, and nerve function. The exam checks a person's mental status, coordination, ability to walk normally, and how well the muscles, senses, and reflexes work. This may also be called a neuro exam or a neurologic exam.

  • Slit-lamp eye exam: An exam that uses a special microscope with a bright, narrow slit of light to check the outside and inside of the eye.

  • 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 brain and spinal cord. A substance called gadolinium is injected into the patient through a vein. The gadolinium collects around the cancer cells so they show up brighter in the picture. This procedure is also called nuclear magnetic resonance imaging (NMRI).

  • PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive 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 do.

  • Lumbar puncture: A procedure used to collect cerebrospinal fluid (the fluid in the spaces around the brain and spinal cord) 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. Tests to diagnose primary CNS lymphoma may include checking the protein level and for signs of cancer in the cerebrospinal fluid.

  • Stereotactic biopsy: A biopsy procedure that uses a computer and a 3-dimensional (3-D) scanning device to find a tumor site and guide the removal of tissue so it can be viewed under a microscope to check for signs of cancer.

The following tests may be done on the samples of tissue that are removed:

  • Flow cytometry: A laboratory test that measures the number of cells in a sample, the percentage of live cells in a sample, and certain characteristics of cells, such as size, shape, and the presence of tumor markers on the cell surface. The cells are stained with a light-sensitive dye, placed in a fluid, and passed in a stream before a laser or other type of light. The measurements are based on how the light-sensitive dye reacts to the light.

  • Immunohistochemistry: A test that uses antibodies to check for certain antigens in a sample of tissue. The antibody is usually linked to a radioactive substance or a dye that causes the tissue to light up under a microscope. This type of test may be used to tell the difference between different types of cancer.

  • Cytogenetic analysis: A laboratory test in which cells in a sample of tissue are viewed under a microscope to look for certain changes in the chromosomes. Other tests, such as fluorescence in situ hybridization (FISH), may also be done to look for certain changes in the chromosomes.

  • Complete blood count (CBC) with differential: A procedure in which a sample of blood is drawn and checked for the following:

    • The number of red blood cells and platelets.

    • The number and type of white blood cells.

    • The amount of hemoglobin (the protein that carries oxygen) in the red blood cells.

    • The portion of the blood 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 makes it.

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

The prognosis (chance of recovery) depends on the following:

  • The patient's age and general health.

  • The level of certain substances in the blood and cerebrospinal fluid (CSF).

  • Where the tumor is in the central nervous system.

  • Whether the patient has AIDS.

Treatment options depend on the following:

  • The stage of the cancer.

  • Where the tumor is in the central nervous system.

  • The patient's age and general health.

  • Whether the cancer has just been diagnosed or has recurred (come back).

Treatment of primary CNS lymphoma works best when the tumor has not spread outside the cerebrum (the largest part of the brain) and the patient is younger than 60 years, able to carry out most daily activities, and does not have AIDS or other diseases that weaken the immune system.

Staging Primary CNS Lymphoma
After primary central nervous system (CNS) lymphoma has been diagnosed, tests are done to find out if cancer cells have spread within the brain and spinal cord or to other parts of the body.

When primary CNS lymphoma continues to grow, it usually does not spread beyond the central nervous system or the eye. The process used to find out if cancer has spread is called staging. It is important to know if cancer has spread to other parts of the body in order to plan treatment. The following tests and procedures may be used in the staging process:

  • 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. For primary CNS lymphoma, a CT scan is done of the chest, abdomen, and pelvis (the part of the body between the hips).

  • PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive 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 do. A PET scan and CT scan may be done at the same time. This is called a PET-CT.

  • 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).

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

There are three ways that cancer spreads in the body.

Cancer can spread through tissue, the lymph system, and the blood:

  • Tissue. The cancer spreads from where it began by growing into nearby areas.

  • Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body.

  • Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body.

Cancer may spread from where it began to other parts of the body.

When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood.

  • Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body.

  • Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body.

The metastatic tumor is the same type of cancer as the primary tumor. For example, if primary CNS lymphoma spreads to the liver, the cancer cells in the liver are actually lymphoma cells. The disease is metastatic CNS lymphoma, not liver cancer.

There is no standard staging system for primary CNS lymphoma.

Treatment Option Overview
There are different types of treatment for patients with primary CNS lymphoma.

Different types of treatment are available for patients with primary central nervous system (CNS) lymphoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. 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. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.

Surgery is not used to treat primary CNS lymphoma.

Three standard treatments 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 or keep them from growing. 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 of cancer being treated.

High-dose radiation therapy to the brain can damage healthy tissue and cause disorders that can affect thinking, learning, problem solving, speech, reading, writing, and memory. Clinical trials have tested the use of chemotherapy alone or before radiation therapy to reduce the damage to healthy brain tissue that occurs with the use of radiation therapy.

Chemotherapy

Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them 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 cerebrospinal fluid (intrathecal chemotherapy), an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type of cancer being treated. Primary CNS lymphoma may be treated with intrathecal chemotherapy and/or intraventricular chemotherapy, in which anticancer drugs are placed into the ventricles (fluid -filled cavities) of the brain.

A network of blood vessels and tissue, called the blood-brain barrier, protects the brain from harmful substances. This barrier can also keep anticancer drugs from reaching the brain. In order to treat CNS lymphoma, certain drugs may be used to make openings between cells in the blood-brain barrier. This is called blood-brain barrier disruption. Anticancer drugs infused into the bloodstream may then reach the brain.

Steroid therapy

Steroids are hormones made naturally in the body. They can also be made in a laboratory and used as drugs. Glucocorticoids are steroid drugs that have an anticancer effect in lymphomas.

New types of treatment are being tested in clinical trials.
High-dose chemotherapy with stem cell transplant

High-dose chemotherapy with stem cell transplant 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 re-infused stem cells grow into (and restore) the body's blood cells.

Targeted therapy

Targeted therapy is a type of treatment that uses drugs or other substances to attack cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. Monoclonal antibody therapy is one type of targeted therapy being studied in the treatment of primary CNS lymphoma.

Monoclonal antibody therapy is 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, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells.

Patients may want to think about taking part in a clinical trial.

For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.

Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.

Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.

Patients can enter clinical trials before, during, or after starting their cancer treatment.

Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.

Clinical trials are taking place in many parts of the country.

Follow-up tests may be needed.

Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. This is sometimes called re-staging.

Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.

Adult Hodgkin Lymphoma

Text in this section is excerpted from “Adult Hodgkin Lymphoma Treatment,” National Cancer Institute at the National Institutes of Health (NIH), May 22, 2015.

General Information About Adult Hodgkin Lymphoma
Adult Hodgkin lymphoma is a disease in which malignant (cancer) cells form in the lymph system.

Adult Hodgkin lymphoma is a type of cancer that develops in the lymph system, part of the body's immune system.

The lymph system 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 lymph and store white blood cells that 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 makes 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 breast bone.

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

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

Because lymph tissue is found throughout the body, Hodgkin lymphoma can begin in almost any part of the body and spread to almost any tissue or organ in the body.

Lymphomas are divided into two general types: Hodgkin lymphoma and non-Hodgkin lymphoma.

Hodgkin lymphoma can occur in both adults and children; however, treatment for adults may be different than treatment for children. Hodgkin lymphoma may also occur in patients who have acquired immunodeficiency syndrome (AIDS); these patients require special treatment.

Hodgkin lymphoma in pregnant women is the same as the disease in non-pregnant women of childbearing age. However, treatment is different for pregnant women.

There are two main types of Hodgkin lymphoma: classical and nodular lymphocyte-predominant.

Most Hodgkin lymphomas are the classical type. The classical type is broken down into the following four subtypes:

  • Nodular sclerosing Hodgkin lymphoma.

  • Mixed cellularity Hodgkin lymphoma.

  • Lymphocyte depletion Hodgkin lymphoma.

  • Lymphocyte-rich classical Hodgkin lymphoma.

Age, gender, and Epstein-Barr infection can affect the risk of adult Hodgkin lymphoma.

Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for adult Hodgkin lymphoma include the following:

  • Being in young or late adulthood.

  • Being male.

  • Being infected with the Epstein-Barr virus.

  • Having a first-degree relative (parent, brother, or sister) with Hodgkin lymphoma.

Pregnancy is not a risk factor for Hodgkin lymphoma.

Signs of adult Hodgkin lymphoma include swollen lymph nodes, fever, night sweats, and weight loss.

These and other signs and symptoms may be caused by adult Hodgkin lymphoma or by other conditions. Check with your doctor if any of the following do not go away:

  • Painless, swollen lymph nodes in the neck, underarm, or groin.

  • Fever for no known reason.

  • Drenching night sweats.

  • Weight loss for no known reason.

  • Itchy skin.

  • Feeling very tired.

Tests that examine the lymph nodes are used to detect (find) and diagnose adult Hodgkin 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's past illnesses and treatments will also be taken.

  • Complete blood count (CBC): 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 makes it.

  • Sedimentation rate: A procedure in which a sample of blood is drawn and checked for the rate at which the red blood cells settle to the bottom of the test tube.

  • Lymph node biopsy: The removal of all or part of a lymph node. 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.

A pathologist views the tissue under a microscope to look for cancer cells, especially Reed-Sternberg cells. Reed-Sternberg cells are common in classical Hodgkin lymphoma.

The following test may be done on tissue that was removed:

  • Immunophenotyping: A laboratory test used to identify cells, based on the types of antigens or markers on the surface of the cell. This test is used to diagnose the specific type of lymphoma by comparing the cancer cells to normal cells of the immune system.

Fig 32.1 Reed-Sternberg cell. Reed-Sternberg cells are large, abnormal lymphocytes that may contain more than one nucleus. These cells are found in Hodgkin lymphoma

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

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

  • The patient's signs and symptoms.

  • The stage of the cancer.

  • The type of Hodgkin lymphoma.

  • Blood test results.

  • The patient's age, gender, and general health.

  • Whether the cancer is recurrent or progressive.

For Hodgkin lymphoma during pregnancy, treatment options also depend on:

  • The wishes of the patient.

  • The age of the fetus.

Adult Hodgkin lymphoma can usually be cured if found and treated earl

Stages of Adult Hodgkin Lymphoma
After adult Hodgkin 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 if cancer has 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 in order to plan treatment. The following tests and procedures may be used in the staging process:

  • 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. For adult Hodgkin lymphoma, CT scans of the neck, chest, abdomen, and pelvis are taken.

  • PET-CT scan: A procedure that combines the pictures from a positron emission tomography (PET) scan and a computed tomography (CT) scan. The PET and CT scans are done at the same time on the same machine. The pictures from both scans are combined to make a more detailed picture than either test would make by itself. A PET scan is a procedure to find malignant tumor cells in the body. A small amount of radioactive 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 do.

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

For pregnant women with Hodgkin lymphoma, staging tests that protect the fetus from the harms of radiation are used. These include:

  • 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).

  • Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram.

There are three ways that cancer spreads in the body.

Cancer can spread through tissue, the lymph system, and the blood:

  • Tissue. The cancer spreads from where it began by growing into nearby areas.

  • Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body.

  • Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body.

Stages of adult Hodgkin lymphoma may include A, B, E, and S.

Adult Hodgkin lymphoma may be described as follows:

  • A: The patient does not have B symptoms (fever, weight loss, or night sweats).

  • B: The patient has B symptoms.

  • E: Cancer is found in an organ or tissue that is not part of the lymph system but which may be next to an involved area of the lymph system.

  • S: Cancer is found in the spleen.

The following stages are used for adult Hodgkin lymphoma:
Stage I

Stage I is divided into stage I and stage IE.

  • Stage I: Cancer is found in one of the following places in the lymph system:

    • One or more lymph nodes in one lymph node group.

    • Waldeyer's ring.

    • Thymus.

    • Spleen.

  • Stage IE: Cancer is found outside the lymph system in one organ or area.

Stage II

Stage II is divided into stage II and stage IIE.

  • Stage II: Cancer is found in two or more lymph node groups either above or below the diaphragm (the thin muscle below the lungs that helps breathing and separates the chest from the abdomen).

  • Stage IIE: Cancer is found in one or more lymph node groups either above or below the diaphragm and outside the lymph nodes in a nearby organ or area.

Stage III

Stage III is divided into stage III, stage IIIE, stage IIIS, and stage IIIE,S.

  • Stage III: Cancer is found in lymph node groups above and below the diaphragm (the thin muscle below the lungs that helps breathing and separates the chest from the abdomen).

  • Stage IIIE: Cancer is found in lymph node groups above and below the diaphragm and outside the lymph nodes in a nearby organ or area.

  • Stage IIIS: Cancer is found in lymph node groups above and below the diaphragm, and in the spleen.

  • Stage IIIE,S: Cancer is found in lymph node groups above and below the diaphragm, outside the lymph nodes in a nearby organ or area, and in the spleen.

Stage IV

In stage IV, the cancer:

  • is found outside the lymph nodes throughout one or more organs, and may be in lymph nodes near those organs; or

  • is found outside the lymph nodes in one organ and has spread to areas far away from that organ; or

  • is found in the lung, liver, bone marrow, or cerebrospinal fluid (CSF). The cancer has not spread to the lung, liver, bone marrow, or CSF from nearby areas.

Adult Hodgkin lymphoma may be grouped for treatment as follows:
Early Favorable

Early favorable adult Hodgkin lymphoma is stage I or stage II, without risk factors.

Early Unfavorable

Early unfavorable adult Hodgkin lymphoma is stage I or stage II with one or more of the following risk factors:

  • A tumor in the chest that is larger than 1/3 of the width of the chest or at least 10 centimeters.

  • Cancer in an organ other than the lymph nodes.

  • A high sedimentation rate (in a sample of blood, the red blood cells settle to the bottom of the test tube more quickly than normal).

  • Three or more lymph nodes with cancer.

  • Symptoms such as fever, weight loss, or night sweats.

Advanced Favorable

Advanced favorable adult Hodgkin lymphoma is stage III or stage IV with three or fewer of the following risk factors:

  • Being male.

  • Being aged 45 years or older.

  • Having stage IV disease.

  • Having a low blood albumin (protein) level (below 4).

  • Having a low hemoglobin level (below 10.5).

  • Having a high white blood cell count (15,000 or higher).

  • Having a low lymphocyte count (below 600 or less than 8% of the white blood cell count).

Advanced Unfavorable

Advanced unfavorable Hodgkin lymphoma is stage III or stage IV with four or more of the following risk factors:

  • Being male.

  • Being aged 45 years or older.

  • Having stage IV disease.

  • Having a low blood albumin (protein) level (below 4).

  • Having a low hemoglobin level (below 10.5).

  • Having a high white blood cell count (15,000 or higher).

  • Having a low lymphocyte count (below 600 or less than 8% of the white blood cell count).

Treatment Option Overview
There are different types of treatment for patients with adult Hodgkin lymphoma.

Different types of treatment are available for patients with adult Hodgkin lymphoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. 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. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.

For pregnant women with Hodgkin lymphoma, treatment is carefully chosen to protect the fetus. Treatment decisions are based on the mother's wishes, the stage of the Hodgkin lymphoma, and the age of the fetus. The treatment plan may change as the signs and symptoms, cancer, and pregnancy change. Choosing the most appropriate cancer treatment is a decision that ideally involves the patient, family, and health care team.

Patients with Hodgkin lymphoma should have their treatment planned by a team of health care providers with expertise in treating lymphomas.

Treatment will be overseen by a medical oncologist, a doctor who specializes in treating cancer. The medical oncologist may refer you to other health care providers who have experience and expertise in treating adult Hodgkin lymphoma and who specialize in certain areas of medicine. These may include the following specialists:

  • Neurosurgeon.

  • Neurologist.

  • Rehabilitation specialist.

  • Radiation oncologist.

  • Endocrinologist.

  • Hematologist.

  • Other oncology specialists.

Patients may develop late effects that appear months or years after their treatment for Hodgkin lymphoma.

Treatment with chemotherapy and/or radiation therapy for Hodgkin lymphoma may increase the risk of second cancers and other health problems for many months or years after treatment. These late effects depend on the type of treatment and the patient's age when treated, and may include:

  • Acute myelogenous leukemia.

  • Cancer of the breast, bone, cervix, gastrointestinal tract, head and neck, lung, soft tissue, and thyroid.

  • Heart, lung, and thyroid disease.

  • Avascular necrosis of bone (death of bone cells caused by lack of blood flow).

  • Herpes zoster (shingles) or severe infection.

  • Depression and fatigue.

  • Infertility.

  • Hypogonadism (low levels of testosterone and estrogen).

Regular follow-up by doctors who are expert in finding and treating late effects is important for the long-term health of patients treated for Hodgkin lymphoma.

Three types of standard treatment are used:
Chemotherapy

Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them 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 cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. Combination chemotherapy is treatment with more than one anticancer drug.

When a pregnant woman is treated with chemotherapy for Hodgkin lymphoma, it isn't possible to protect the fetus from being exposed to the chemotherapy. Some chemotherapy regimens may cause birth defects if given in the first trimester. Vinblastine is an anticancer drug that has not been linked with birth defects when given in the second half of pregnancy.

Radiation therapy

Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. 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.

For a pregnant woman with Hodgkin lymphoma, radiation therapy should be postponed until after delivery, if possible, to avoid any risk to the fetus. If immediate treatment is needed, the woman may decide to continue the pregnancy and receive radiation therapy. However, lead used to shield the fetus may not protect it from scattered radiation that could possibly cause cancer in the future.

Surgery

Laparotomy is a procedure in which an incision (cut) is made in the wall of the abdomen to check the inside of the abdomen for signs of disease. The size of the incision depends on the reason the laparotomy is being done. Sometimes organs are removed or tissue samples are taken and checked under a microscope for signs of disease. If cancer is found, the tissue or organ is removed during the laparotomy.

For pregnant patients with Hodgkin lymphoma, treatment options also include:
Watchful waiting

Watchful waiting is closely monitoring a patient's condition without giving any treatment unless signs or symptoms appear or change. Delivery may be induced when the fetus is 32 to 36 weeks old, so that the mother can begin treatment.

Steroid therapy

Steroids are hormones made naturally in the body by the adrenal glands and by reproductive organs. Some types of steroids are made in a laboratory. Certain steroid drugs have been found to help chemotherapy work better and help stop the growth of cancer cells. Steroids can also help the lungs of the fetus develop faster than normal. This is important when delivery is induced early.

New types of treatment are being tested in clinical trials.
Chemotherapy and radiation therapy with stem cell transplant

High-dose chemotherapy and radiation therapy with stem cell transplant is a way of giving high doses of chemotherapy and radiation therapy 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 therapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These re-infused stem cells grow into (and restore) the body's blood cells. The use of lower-dose chemotherapy and radiation therapy with stem cell transplant is also being studied.

Monoclonal antibody therapy

Monoclonal antibody therapy is 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, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells.

Patients may want to think about taking part in a clinical trial.

For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.

Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.

Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.

Patients can enter clinical trials before, during, or after starting their cancer treatment.

Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.

Clinical trials are taking place in many parts of the country.

Follow-up tests may be needed.

Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. This is sometimes called re-staging.

Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.

Adult Non-Hodgkin Lymphoma

Text in this section is excerpted from “Adult Non Hodgkin Lymphoma Treatment,” National Cancer Institute at the National Institutes of Health (NIH), April 25, 2014.

General Information About Adult Non-Hodgkin Lymphoma
Adult non-Hodgkin 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 lymph and store white blood cells that 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 makes lymphocytes, filters the blood, stores blood cells, and destroys old blood cells. It is 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 breast bone.

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

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

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

Non-Hodgkin lymphoma in pregnant women is the same as the disease in non-pregnant women of childbearing age. However, treatment is different for pregnant women.

Non-Hodgkin 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 lymphoma and non-Hodgkin lymphoma.

Waldenström macroglobulinemia is a type of non-Hodgkin lymphoma.

Waldenström macroglobulinemia begins in a type of white blood cell called B lymphocytes. Certain B lymphocytes multiply out of control and make large amounts of a protein called monoclonal immunoglobulin M (IgM) antibody. High levels of IgM in the blood cause the blood to thicken and leads to many of the symptoms of Waldenström macroglobulinemia. Waldenström macroglobulinemia is also called lymphoplasmacytic lymphoma.

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

Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for adult non-Hodgkin lymphoma include the following:

  • Being older, male, or white.

  • Having one of the following medical conditions:

    • An Inherited immune disorder (for example, hypogammaglobulinemia or Wiskott-Aldrich syndrome)

    • An autoimmune disease (for example, rheumatoid arthritis, psoriasis, or Sjögren syndrome).

    • 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 lymphoma.

Signs and symptoms of adult non-Hodgkin lymphoma include fever, sweating, weight loss, and fatigue.

These and other signs and symptoms may be caused by adult non-Hodgkin lymphoma or by other conditions. Check with your doctor if you have any of the following:

  • Painless swelling in the lymph nodes in the neck, underarm, groin, or stomach.

  • Fever for no known reason.

  • Drenching night sweats.

  • Feeling very tired.

  • Weight loss for no known reason.

  • Skin rash or itchy skin.

  • Pain in the chest, abdomen, or bones for no known reason.

Signs and symptoms of Waldenström macroglobulinemia depend on the part of the body affected. Most patients with Waldenström macroglobulinemia have no signs or symptoms. Check with your doctor if you have any of the following:

  • Feeling very tired.

  • Headache.

  • Easy bruising or bleeding, such as nosebleeds or bleeding from the gums.

  • Vision changes, such as blurred vision or blind spots.

  • Dizziness.

  • Pain, tingling, or numbness, especially in the hands, feet, fingers, or toes.

  • Confusion.

  • Pain or a feeling of fullness below the ribs on the left side.

  • Painless lumps in the neck, underarm, stomach, or groin.

  • Weight loss for no known reason.

Tests that examine the body and lymph system are used to help detect (find) and diagnose adult non-Hodgkin 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's health habits and past illnesses and treatments will also be taken.

  • Blood and urine immunoglobulin studies: A procedure in which a blood or urine sample is checked to measure the amounts of certain antibodies (immunoglobulins). In Waldenström macroglobulinemia, immunoglobulin M (IgM) and beta-2-microglobulin is measured. A higher- or lower-than-normal amount of these substances can be a sign of disease.

  • Blood viscosity test: A procedure in which a blood sample is checked to see how “thick” the blood is. In Waldenström macroglobulinemia, when the amount of monoclonal immunoglobulin M (IgM) antibody in the blood becomes very high, the blood thickens and may cause signs or symptoms.

  • Flow cytometry: A laboratory test that measures the number of cells in a sample, the percentage of live cells in a sample, and certain characteristics of cells, such as size, shape, and the presence of tumor markers on the cell surface. The cells are stained with a light-sensitive dye, placed in a fluid, and passed in a stream before a laser or other type of light. The measurements are based on how the light-sensitive dye reacts to the light. This test is used to diagnose Waldenström macroglobulinemia.

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

  • 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.

    • Fine-needle aspiration (FNA) biopsy: The removal of tissue or fluid using a thin needle.

    • Laparoscopy: A surgical procedure to look at the organs inside the abdomen to check for signs of disease. Small incisions (cuts) are made in the wall of the abdomen and a laparoscope (a thin, lighted tube) is inserted into one of the incisions. Other instruments may be inserted through the same or other incisions to perform procedures such as removing organs or taking tissue samples to be checked under a microscope for signs of disease.

    • Laparotomy: A surgical procedure in which an incision (cut) is made in the wall of the abdomen to check the inside of the abdomen for signs of disease. The size of the incision depends on the reason the laparotomy is being done. Sometimes organs are removed or tissue samples are taken and checked under a microscope for signs of disease.

If cancer is found, the following tests may be done to study the cancer cells:

  • Immunohistochemistry: A test that uses antibodies to check for certain antigens in a sample of tissue. The antibody is usually linked to a radioactive substance or a dye that causes the tissue to light up under a microscope. This type of test may be used to tell the difference between different types of cancer.

  • Cytogenetic analysis: A laboratory test in which cells in a sample of tissue are viewed under a microscope to look for certain changes in the chromosomes.

  • Immunophenotyping: A process used to identify cells, based on the types of antigens or markers on the surface of the cell. This process is used to diagnose specific types of leukemia and lymphoma by comparing the cancer cells to normal cells of the immune system.

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 lymphoma.

  • The amount of lactate dehydrogenase (LDH) in the blood.

  • The amount of beta-2-microglobulin in the blood (for Waldenström macroglobulinemia).

  • The patient's age and general health.

  • Whether the lymphoma has just been diagnosed or has recurred (come back).

For non-Hodgkin lymphoma during pregnancy, the treatment options also depend on:

  • The wishes of the patient

  • Which trimester of pregnancy the patient is in.

Some types of non-Hodgkin lymphoma spread more quickly than others do. Most non-Hodgkin lymphomas that occur during pregnancy are aggressive. Delaying treatment of aggressive lymphoma until after the baby is born may lessen the mother's chance of survival. Immediate treatment is often recommended, even during pregnancy.

Stages of Adult Non-Hodgkin Lymphoma
After adult non-Hodgkin 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:

  • Complete blood count (CBC) with differential: A procedure in which a sample of blood is drawn and checked for the following:

    • The number of red blood cells and platelets.

    • The number and type of white blood cells.

    • The amount of hemoglobin (the protein that carries oxygen) in the red blood cells.

    • The portion of the blood 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 makes it.

  • CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the lung, lymph nodes, and liver, 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 radioactive 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 do.

  • 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).

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

For pregnant women with non-Hodgkin lymphoma, staging tests that protect the fetus from the harms of radiation are used. These include MRI, bone marrow aspiration and biopsy, lumbar puncture, and ultrasound, which do not use radiation. An ultrasound exam is a procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram.

There are three ways that cancer spreads in the body.

Cancer can spread through tissue, the lymph system, and the blood:

  • Tissue. The cancer spreads from where it began by growing into nearby areas.

  • Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body.

  • Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body.

Stages of adult non-Hodgkin lymphoma may include E and S.

Adult non-Hodgkin lymphoma may be described as follows:

  • E: “E” stands for extranodal and means the cancer is found in an area or organ other than the lymph nodes or has spread to tissues beyond, but near, the major lymphatic areas.

  • S: “S” stands for spleen and means the cancer is found in the spleen.

The following stages are used for adult non-Hodgkin lymphoma:
Stage I

Stage I adult non-Hodgkin lymphoma is divided into stage I and stage IE.

  • Stage I: Cancer is found in one lymphatic area (lymph node group, tonsils and nearby tissue, thymus, or spleen).

  • Stage IE: Cancer is found in one organ or area outside the lymph nodes.

Stage II

Stage II adult non-Hodgkin lymphoma is divided into stage II and stage IIE.

  • Stage II: Cancer is found in two or more lymph node groups either above or below the diaphragm (the thin muscle below the lungs that helps breathing and separates the chest from the abdomen).

  • Stage IIE: Cancer is found in one or more lymph node groups either above or below the diaphragm. Cancer is also found outside the lymph nodes in one organ or area on the same side of the diaphragm as the affected lymph nodes.

Stage III

Stage III adult non-Hodgkin lymphoma is divided into stage III, stage IIIE, stage IIIS, and stage IIIE+S.

  • Stage III: Cancer is found in lymph node groups above and below the diaphragm (the thin muscle below the lungs that helps breathing and separates the chest from the abdomen).

  • Stage IIIE: Cancer is found in lymph node groups above and below the diaphragm and outside the lymph nodes in a nearby organ or area.

  • Stage IIIS: Cancer is found in lymph node groups above and below the diaphragm, and in the spleen.

  • Stage IIIE+S: Cancer is found in lymph node groups above and below the diaphragm, outside the lymph nodes in a nearby organ or area, and in the spleen.

Stage IV

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

  • is found throughout one or more organs that are not part of a lymphatic area (lymph node group, tonsils and nearby tissue, thymus, or spleen), and may be in lymph nodes near those organs; or

  • is found in one organ that is not part of a lymphatic area and has spread to organs or lymph nodes far away from that organ; or

  • is found in the liver, bone marrow, cerebrospinal fluid (CSF), or lungs (other than cancer that has spread to the lungs from nearby areas).

Adult non-Hodgkin lymphomas are also described based on how fast they grow and where the affected lymph nodes are in the body.

Indolent or aggressive:

  • Indolent lymphomas: These tend to grow and spread slowly and have few symptoms.

  • Aggressive lymphomas: These grow and spread quickly and have severe symptoms. Lymphoblastic lymphoma, diffuse small non-cleaved cell lymphoma /Burkitt lymphoma, and mantle cell lymphoma are three types of aggressive adult non-Hodgkin lymphoma. Aggressive lymphomas are seen more often in patients who are HIV -positive (AIDS -related lymphoma).

Contiguous or noncontiguous:

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

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

There is no standard staging system for Waldenström macroglobulinemia.

Treatment Options for Non-Hodgkin Lymphoma
Indolent, Stage I and Contiguous Stage II Adult Non- Hodgkin Lymphoma

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

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

  • Watchful waiting.

  • Chemotherapy with radiation therapy.

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

  • Monoclonal antibody therapy with or without chemotherapy.

  • Treatments used for more advanced disease, in patients who can't be treated with radiation therapy.

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

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

  • Combination chemotherapy with or without radiation therapy to areas where cancer is found.

  • A clinical trial of monoclonal antibody therapy and combination chemotherapy with steroids. Radiation therapy may also be given.

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

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

  • Watchful waiting for patients who do not have symptoms.

  • Chemotherapy with or without steroids.

  • Combination chemotherapy with steroids.

  • Monoclonal antibody therapy with or without chemotherapy.

  • Radiolabeled monoclonal antibody therapy.

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

  • A clinical trial of chemotherapy with or without total-body irradiation (radiation therapy to the entire body) or radiolabeled monoclonal antibody therapy, followed by autologous or allogeneic stem cell transplant.

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

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

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

  • Combination chemotherapy with radiation therapy or monoclonal antibody therapy.

  • Combination chemotherapy with CNS prophylaxis.

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

Diffuse Small Non-cleaved Cell / Burkitt Lymphoma

Treatment of adult diffuse small non-cleaved 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 transplant.

Waldenström Macroglobulinemia

Treatment of Waldenström macroglobulinemia may include the following:

  • Watchful waiting.

  • Plasmapheresis and chemotherapy.

  • Combinations of chemotherapy using one or more anticancer drugs and targeted therapy with amonoclonal antibody or a proteasome inhibitor.

  • Biologic therapy with interferon.

  • A clinical trial of stem cell transplant.

Recurrent Adult Non-Hodgkin Lymphoma
Indolent, Recurrent Adult Non-Hodgkin Lymphoma

Treatment of indolent, recurrent adult non-Hodgkin 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 transplant.

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

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

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

  • A clinical trial of monoclonal antibody therapy.

  • A clinical trial of radiolabeled monoclonal antibody therapy.

Aggressive, Recurrent Adult Non-Hodgkin Lymphoma

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

  • Stem cell transplant.

  • Monoclonal antibody therapy with or without combination chemotherapy followed by autologous stem cell transplant.

  • Radiolabeled monoclonal antibody therapy.

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

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

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

  • Chemotherapy.

  • Palliative therapy with low-dose radiation therapy to relieve symptoms and improve quality of life.

Non-Hodgkin Lymphoma During Pregnancy
Aggressive Non-Hodgkin Lymphoma During the First Trimester of Pregnancy

When aggressive non-Hodgkin lymphoma is diagnosed in the first trimester of pregnancy, medical oncologists may advise the patient to end her pregnancy so that treatment may begin. Treatment is usually chemotherapy with or without radiation therapy.

Aggressive Non-Hodgkin Lymphoma During the Second and Third Trimesters of Pregnancy

When possible, treatment should delayed until after an early delivery, so that the anticancer drugs or radiation therapy will not affect the fetus. However, sometimes the cancer will need to be treated right away to increase the mother's chance of survival.

Indolent Non-Hodgkin Lymphoma During Pregnancy

Women who have indolent (slow-growing) non-Hodgkin lymphoma can usually delay treatment with watchful waiting.

© 2015 Omnigraphics, Inc.

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