Lymphoma - HodgkinLast Updated: July 27, 2011 This section has been reviewed and approved by the Cancer.Net Editorial Board, 04/11 Overview
Hodgkin lymphoma, also called Hodgkin’s disease, is one category of lymphoma, a cancer of the lymph system. Lymphoma begins when cells in the lymph system change and grow uncontrollably, which may form a tumor. About the lymph system The lymph system is made up of thin tubes that branch out to all parts of the body. Its job is to fight infection and disease. The lymph system carries lymph, a colorless fluid containing lymphocytes (white blood cells). Lymphocytes fight germs in the body. B-lymphocytes (also called B cells) make antibodies to fight bacteria, and T-lymphocytes (also called T cells) kill viruses and foreign cells and trigger the B cells to make antibodies. Types of Hodgkin Lymphoma There are different types of Hodgkin lymphoma. It is important to know the type, as this may affect the choice of treatment. Doctors determine the type of Hodgkin lymphoma based on how the cells in a tissue sample look under a microscope and whether the cells contain abnormal patterns of certain proteins. There is another type of Hodgkin lymphoma that is not a part of the CHL group; rather, it is more similar at the protein and genetic level to B-cell non-Hodgkin lymphoma. Find out more about basic cancer terms used in this section. Looking for More of an Overview? If you would like additional introductory information, explore the following items on Cancer.Net:
Or, choose “Next” (below, right) to continue reading this detailed section. To select a specific topic within this section, use the icon panel located on the right side of your screen. Statistics
This year, an estimated 8,830 people (4,820 men and 4,010 women) in the United States will be diagnosed with Hodgkin lymphoma. It is estimated that 1,300 deaths (760 men and 540 women) from this disease will occur this year. Hodgkin lymphoma affects both children and adults. It is most common in two age groups: ages 15 to 40 (particularly young adults in their 20s) and after 55. The one-year relative survival rate (the percentage of people who survive at least one year after the cancer is detected, excluding those who die from other diseases) of patients with Hodgkin lymphoma is 92%. The five-year and ten-year relative survival rates are 85% and 81%, respectively. Cancer survival statistics should be interpreted with caution. These estimates are based on data from thousands of people with this type of cancer in the United States, but the actual risk for a particular individual may differ. It is not possible to tell a person how long he or she will live with Hodgkin lymphoma. Because the survival statistics are measured in multi-year intervals, they may not represent advances made in the treatment or diagnosis of this cancer. Learn more about understanding statistics. Statistics adapted from the American Cancer Society’s publication, Cancer Facts & Figures 2011. Medical Illustrations Risk Factors
A risk factor is anything that increases a person’s chance of developing cancer. Although risk factors can influence the development of cancer, most do not directly cause cancer. Some people with several risk factors never develop cancer, while others with no known risk factors do. However, knowing your risk factors and talking about them with your doctor may help you make more informed lifestyle and health care choices. The exact cause of Hodgkin lymphoma is not known, but the following factors may raise a person’s risk of developing Hodgkin lymphoma: It is important to note that, although viruses may be involved in the development of Hodgkin lymphoma, there is no evidence that this type of cancer is contagious. Close contact with someone with Hodgkin lymphoma does not increase a person’s risk of developing the disease. Symptoms and Signs
People with Hodgkin lymphoma may experience the following symptoms or signs. Sometimes, people with Hodgkin lymphoma do not show any of these symptoms, or these symptoms may be caused by a medical condition that is not cancer. If you are concerned about a symptom or sign on this list, please talk with your doctor. Common symptoms associated with Hodgkin lymphoma may include:
If the lymph nodes in the chest are affected, they may press on the windpipe and cause shortness of breath, cough, or chest discomfort. The doctor may use certain symptoms to help describe the disease, called staging. Each stage may be subdivided into "A" and "B" categories.
Your doctor will ask you questions about the symptoms you are experiencing to help find out the cause of the problem, called a diagnosis. This may include how long you’ve been experiencing the symptom(s) and how often. Diagnosis
Doctors use many tests to diagnose cancer and find out the extent of the disease. Some tests may also determine which treatments may be the most effective. For most types of cancer, a biopsy is the only way to make a definitive diagnosis. If a biopsy is not possible, the doctor may suggest other tests that will help make a diagnosis. Imaging tests may be used to find out whether the cancer has spread. Your doctor may consider these factors when choosing a diagnostic test:
The following tests may be used to diagnose Hodgkin lymphoma: Medical history and physical examination. A thorough medical history and physical examination can show evidence of typical symptoms, such as night sweats and fevers and affected or enlarged lymph nodes or spleen. Biopsy. A biopsy is the removal of a small amount of tissue for examination under a microscope. Other tests can suggest that cancer is present, but the diagnosis of Hodgkin lymphoma can only be made after a biopsy of an affected piece of tissue. Most commonly, this will be a lymph node in the neck, under the arm, or in the groin. If there are no lymph nodes in these areas, a biopsy of other lymph nodes, such as those in the center of the chest, may be necessary. This type of biopsy usually requires minor surgery, although occasionally it is possible to do a biopsy using a needle under local anesthesia while the patient is undergoing a scan, most commonly a computed tomography (CT or CAT) scan (see below). The CT scan is used to help guide the doctor doing the biopsy to the appropriate place. Computed tomography (CT or CAT) scan. A CT scan creates a three-dimensional picture of the inside of the body with an x-ray machine. A computer then combines these images into a detailed, cross-sectional view that shows any abnormalities or tumors. Sometimes, a contrast medium (special dye) is injected into a patient’s vein to provide better detail. A CT scan of the chest and abdomen can help find cancer that has spread to the lungs, lymph nodes, and liver. Magnetic resonance imaging (MRI). An MRI uses magnetic fields, not x-rays, to produce detailed images of the body. A contrast medium may be injected into a patient’s vein to create a clearer picture. This is sometimes used in Hodgkin lymphoma. Bone marrow biopsy and aspiration. These two procedures are similar and often done at the same time. Bone marrow (the soft, spongy tissue that is found inside the center of bones) has both a solid and a liquid part. A bone marrow biopsy is the removal of a small amount of solid tissue using a needle. An aspiration removes a sample of fluid with a needle. The sample(s) are then analyzed by a pathologist. Lymphoma often spreads to the bone marrow, so looking at a sample of the bone marrow can be important for doctors to diagnose lymphoma and determine the stage. The aspirate is also used to identify any chromosome abnormalities. The decision regarding the need for a bone marrow biopsy depends on the extent of the disease and the results of laboratory tests. Learn more about what to expect when having common tests, procedures, and scans. After these diagnostic tests are done, your doctor will review all of the results with you. If the diagnosis is cancer, these results also help the doctor describe the cancer; this is called staging. Learn more about the first steps to take after a diagnosis of cancer. Staging
Staging helps to define where the Hodgkin lymphoma is located, if or where it has spread, and whether it is affecting the functions of other organs in the body. Doctors use diagnostic tests to determine the cancer’s stage, so staging may not be complete until all tests are finished. Knowing the stage helps the doctor to decide what kind of treatment is best and can help predict a patient’s prognosis (chance of recovery). There are different stage descriptions for different types of cancer.
The stage of lymphoma describes the extent of the spread of the tumor, using the terms stage I through IV (one through four). As explained in Symptoms, each stage may also be subdivided into “A” and “B” categories, based on the presence or absence of specific symptoms. Stage II: Either one of these conditions:
Stage III: There is cancer in lymph node areas on both sides of the diaphragm (stage III). In addition, there may be involvement of an extralymphatic organ (stage IIIE), involvement of the spleen (using the letter “S,” stage IIIS), or both (stage IIIES). Stage IV: There is disseminated (multifocal) involvement, meaning that the lymphoma has spread throughout multiple areas. Common sites for disseminated disease include the liver, bone marrow, or lungs. Recurrent: Recurrent lymphoma is lymphoma that comes back after treatment. Lymphoma may return in the area where it first started or in another part of the body. Recurrence may occur shortly after the first treatment or years later. If there is a recurrence, the cancer may need to be staged again (re-staging) using the system above. Prognostic factors. In addition to stage, doctors use other prognostic factors to help plan the best treatment and predict how successful treatment will be. For patients with Hodgkin lymphoma, several factors can predict whether the disease will return and which treatments will be successful. A patient is treated as having high-risk disease or low-risk disease based how many of the following prognostic factors there are. Below are prognostic factors for patients with early-stage Hodgkin lymphoma. In general, the fewer poor prognostic factors a patient has, the longer remission he/she should experience and the more successful treatments should be. Prognosis depends on several factors:
Below are poor prognostic factors for patients with advanced Hodgkin lymphoma.
Used with permission of the AJCC, Chicago, Illinois. The original source for this material is the AJCC Cancer Staging Manual, Seventh Edition (2010) published by Springer-Verlag New York, www.cancerstaging.net. Treatment
This section outlines treatments that are the standard of care (the best proven treatments available) for this specific type of cancer. When making treatment plan decisions, patients are also encouraged to consider clinical trials as an option. A clinical trial is a research study to test a new treatment to evaluate whether it is safe, effective, and possibly better than standard treatment. Your doctor can help you review all treatment options. For more information, see the Clinical Trials and Current Research sections. Descriptions of the most common treatment options for Hodgkin lymphoma are listed below. Treatment options and recommendations depend on several factors, including the type and stage of cancer, possible side effects, and the patient’s preferences and overall health. Learn more about making treatment decisions. Radiation therapy Radiation therapy is the use of high energy x-rays or other particles to kill cancer cells. A doctor who specializes in giving radiation therapy to treat cancer is called a radiation oncologist. Radiation therapy for Hodgkin lymphoma is always external-beam radiation therapy, which is radiation given from a machine outside the body. A radiation therapy regimen (schedule) usually consists of a specific number of treatments given over a set period of time. Whenever possible, radiation therapy is now typically targeted to the affected lymph node areas to reduce the risk of side effects. Chemotherapy Chemotherapy is the use of drugs to kill cancer cells, usually by stopping the cancer cells’ ability to grow and divide. Systemic chemotherapy is delivered through the bloodstream to reach cancer cells throughout the body. Chemotherapy is given by a medical oncologist, a doctor who specializes in treating cancer with medication, or a hematologist, a doctor who specializes in treating blood disorders. A chemotherapy regimen usually consists of a specific number of cycles given over a set period of time. A patient may receive one drug at a time or combinations of different drugs at the same time. Many different types of chemotherapy may be used for Hodgkin lymphoma. The most commonly used combination of drugs in the United States is called "ABVD." Another combination of drugs known as “BEACOPP” is now widely used in Europe and is being used more commonly in the United States.
At the moment, it is unclear which of these chemotherapy treatments is best for patients with Hodgkin lymphoma, and the best treatment may differ depending on the type and stage of the lymphoma. For this reason, many clinical trials are underway comparing these different chemotherapy treatments. These clinical trials are designed to determine which combination is the most effective for the treatment of Hodgkin lymphoma and which has the fewest short-term and long-term side effects. At various times during the course of chemotherapy, your doctors will usually repeat some of the original tests, especially CT scans and PET scans. These tests are used to monitor the lymphoma and see how well it is responding to treatment. The side effects of chemotherapy depend on the individual and the dose used, but they can include fatigue, risk of infection, nausea and vomiting, loss of appetite, and diarrhea. These side effects usually go away once treatment is finished. Although the risk of long-term side effects decreases as treatments improve, chemotherapy sometimes causes late and permanent side effects. Learn more about late effects of treatment. Learn more about chemotherapy and preparing for treatment. The medications used to treat cancer are continually being evaluated. Talking with your doctor is often the best way to learn about the medications prescribed for you, their purpose, and their potential side effects or interactions with other medications. Learn more about your prescriptions by using searchable drug databases. Stem cell transplantation/bone marrow transplantation A stem cell transplant is a medical procedure in which diseased bone marrow is replaced by highly specialized cells, called hematopoietic stem cells. Hematopoietic stem cells are found both in the bloodstream and in the bone marrow. Today, this procedure is more commonly called a stem cell transplant, rather than bone marrow transplant, because it is the blood stem cells that are typically being transplanted, not the actual bone marrow tissue. Before recommending transplantation, doctors will talk with the patient about the risks of this treatment and consider several other factors, such as the type of cancer, results of any previous treatment, and patient’s age and general health. In an AUTO transplant—the type used most commonly for Hodgkin lymphoma—the patient’s own stem cells are used. The stem cells are obtained from the patient when he or she is in remission from previous treatment. The stem cells are then frozen until they are needed, usually after the high-dose treatment (explained below) is completed. In an ALLO transplant, stem cells are obtained from a donor whose tissue matches the patient’s on a genetic level; this testing is called HLA-typing. Most often, a patient’s brother or sister serves as the donor, although unrelated donors can serve as the donor, too. Millions of people worldwide have volunteered to donate stem cells for patients who do not have matched family members; your health care team will search a computer registry to look for a match. In addition, a donation of stem cells derived from umbilical cord blood is sometimes considered if family donors are not available. ALLO transplantation is only used occasionally for Hodgkin lymphoma. In an ALLO transplant, another major risk is that the donor’s cells will recognize the patient’s body as foreign, causing graft-versus-host disease (GVHD). GVHD may be a serious complication of allogeneic transplants and can be fatal. Other side effects may include liver problems, diarrhea, infections, and rashes. However, GVHD can also be a benefit because the donor cells can recognize the cancer cells as foreign and destroy these cells, a mechanism that is one of the major reasons why ALLO transplantation generally works so well over the long term. The risk of GVHD can be reduced with exact HLA-type matching and the use of preventive drugs. In an AUTO transplant, there is little risk of GVHD because the replacement stem cells are the patient’s own cells. However, there is a risk in an autologous transplant that some of the cells that are put back into the patient could still be cancerous. Learn more about bone marrow and stem cell transplantation.Recurrent Hodgkin lymphoma Progressive Hodgkin lymphoma Progressive disease is when the cancer becomes larger or spreads while the patient is being treated for the original lymphoma. However, progressive disease is uncommon for people with Hodgkin lymphoma. Patients with this diagnosis are encouraged to talk with doctors who are experienced in treating this stage of cancer, because there can be different opinions about the best treatment plan. Learn more about seeking a second opinion before starting treatment, so you are comfortable with the treatment plan chosen. This discussion may include clinical trials. Most patients will be advised to receive a stem cell transplantation (see above), which appears to be more effective in treating progressive Hodgkin lymphoma than standard chemotherapy. In addition to treatment to slow, stop, or eliminate the cancer (also called disease-directed treatment), an important part of cancer care is relieving a person’s symptoms and side effects. It includes supporting the patient with his or her physical, emotional, and social needs, an approach called palliative or supportive care. People often receive disease-directed therapy and treatment to ease symptoms at the same time. About Clinical Trials
Doctors and scientists are always looking for better ways to treat patients with Hodgkin lymphoma. To make scientific advances, doctors create research studies involving people, called clinical trials. Many clinical trials are focused on new treatments, evaluating whether a new treatment is safe, effective, and possibly better than the current (standard) treatment. These types of studies evaluate new drugs, different combinations of existing treatments, new approaches to radiation therapy or surgery, and new methods of treatment. Patients who participate in clinical trials are often among the first to receive new treatments before they are widely available. However, there is no guarantee that the new treatment will be safe, effective, or better than a standard treatment. Patients decide to participate in clinical trials for many reasons. For some patients, a clinical trial is the best treatment option available. Because standard treatments are not perfect, patients are often willing to face the added uncertainty of a clinical trial in the hope of a better result. Other patients volunteer for clinical trials because they know that these studies are the only way to make progress in treating Hodgkin lymphoma. Even if they do not benefit directly from the clinical trial, their participation may benefit future patients with Hodgkin lymphoma. To join a clinical trial, patients must participate in a process known as informed consent. During informed consent, the doctor should list all of the patient's options so that the person understands how the new treatment differs from the standard treatment. The doctor must also list all of the risks of the new treatment, which may or may not be different than the risks of standard treatment. Finally, the doctor must explain what will be required of each patient in order to participate in the clinical trial, including the number of doctor visits, tests, and the schedule of treatment. Learn more about clinical trials, including patient safety, phases of a clinical trial, deciding to participate in a clinical trial, questions to ask the research team, and find cancer clinical trials. For specific topics being studied for Hodgkin lymphoma, learn more in the Current Research section. Patients who participate in a clinical trial may stop participating at any time for any personal or medical reason. This may include that the new treatment is not working or there are serious side effects. It is important that patients participating in a clinical trial talk with their doctor and researchers about who will be providing their treatment and care during the clinical trial, after the clinical trials ends, and/or if the patient chooses to leave the clinical trial before it ends. Side Effects
Cancer and its treatment can cause a variety of side effects. However, doctors have made major strides in recent years in reducing pain, nausea and vomiting, and other physical side effects of cancer treatments. Many treatments used today are less intensive but as effective as treatments used in the past. Doctors also have many ways to provide relief to patients when such side effects occur. Fear of treatment side effects is common after a diagnosis of cancer, but it may be helpful to know that preventing and controlling side effects is a major focus of your health care team. Before treatment begins, talk with your doctor about possible side effects of the specific treatments you will be receiving. The specific side effects that can occur depend on a variety of factors, including the type of cancer, its location, the individual treatment plan (including the length and dosage of treatment), and your overall health. Common side effects for each treatment option are described in detail within the Treatment section. Ask your doctor which side effects are most likely to happen (and which are not), when side effects are likely to occur, and how they will be addressed by the health care team. Also, be sure to communicate with the doctor about side effects you experience during and after treatment. Care of a patient’s symptoms and side effects is an important part of a person’s overall treatment plan; this is called palliative or supportive care. It helps people with cancer at any stage of illness be as comfortable as possible. Learn more about the most common side effects of cancer and different treatments, along with ways to prevent or control them. Be sure to talk with your doctor about the level of caregiving you may need during treatment and recovery, as family members and friends often play an important role in the care of a person with Hodgkin lymphoma. Learn more about caregiving. In addition to physical side effects, there may be psychosocial (emotional and social) effects as well. For many patients, a diagnosis of Hodgkin lymphoma is stressful and can bring difficult emotions, including depression and anxiety. Patients and their families are encouraged to share their feelings with a member of their health care team, who can help with coping strategies. Learn more about the importance of addressing such needs, including concerns about managing the cost of your medical care. Learn more about late effects or long-term side effects by reading the Late Effects and After Treatment sections or talking with your doctor. Late Effects of Treatment
Patients treated for Hodgkin lymphoma have an increased risk of developing other diseases or conditions later in life because both chemotherapy and radiation therapy can cause permanent damage. Treatments have improved in the last 30 years, and now patients are less likely to experience late effects, but there is still some risk. Therefore, it is important that patients see their doctors for their follow-up care regularly to monitor any developments.
The risks of secondary cancers are likely to be lower in the future because the types of therapy now used carry fewer risks. After Treatment
After treatment for Hodgkin lymphoma ends, talk with your doctor about developing a follow-up care plan. This plan may include doctor visits and medical tests to monitor your recovery for the coming months and years. Follow-up care after treatment for Hodgkin lymphoma is important to monitor for possible recurrence of the lymphoma and late effects of treatment. The frequency of follow-up care and the tests performed depends on several factors, including the original extent of the Hodgkin lymphoma and type of treatment. Typically, all of the tests—including CT scans, PET scans, and bone marrow biopsies—are repeated after treatment ends to ensure that the lymphoma is gone. Then, the frequency of additional screening depends on the results of the initial set of tests performed after treatment. In general, each follow-up visit includes a discussion with the doctor, physical examination, and blood tests. During some visits, scans are done. At most cancer centers, follow-up visits are initially scheduled every two to three months during the time period with the greatest risk of recurrence, and the interval between visits increases over time. Later visits may only be two to three times per year until five years has passed; then, annual visits should be continued with an oncologist. Special attention should be paid to cancer screening and detection, as well as heart risk factors, for the person’s lifetime. For patients who received radiation therapy to the neck or chest, monitoring thyroid function is important. Follow-up care should also address the person’s quality of life, including emotional concerns. In particular, Hodgkin lymphoma survivors are encouraged to be aware of symptoms of depression and talk with their doctor immediately if they have such symptoms. Patients treated for Hodgkin lymphoma should get an annual flu shot. It may be recommended that some patients get an immunization against pneumonia, which may be repeated every five to seven years. ASCO offers cancer treatment summary forms to help keep track of the cancer treatment you received and develop a survivorship care plan once treatment is completed. People recovering from Hodgkin lymphoma are encouraged to follow established guidelines for good health, such as maintaining a healthy weight, not smoking, eating a balanced diet, and having recommended cancer screening tests. Talk with your doctor to develop a plan that is best for your needs. Moderate physical activity can help rebuild your strength and energy level. Your doctor can help you create an appropriate exercise plan based upon your needs, physical abilities, and fitness level. Learn more about the next steps to take in survivorship, including making positive lifestyle changes. Find out more about common terms used after cancer treatment is complete. Current Research
Doctors are working to learn more about Hodgkin lymphoma, ways to prevent it, how to best treat it, and how to provide the best care to people diagnosed with this disease. The following areas of research may include new options for patients through clinical trials. Always talk with your doctor about the diagnostic and treatment options best for you. Imaging tests. Studies are finished and results are being analyzed on the use of PET and CT scans early during treatment to monitor how the lymphoma is responding to treatment and as a routine screening test after treatment. Some new clinical trials will be opening soon in which changes to chemotherapy treatment may be made depending on the results of a PET scan early in the treatment. Your doctor may ask you to take part in such a study and, if you are interested, will explain it to you in detail. New chemotherapy. New chemotherapy, combinations of chemotherapy, lower doses, and shorter schedules are being studied in clinical trials to reduce short-term side effects and long-term health risks to patients receiving chemotherapy. Gene profiling. Some researchers are looking at the specific genes and proteins that are found in Hodgkin lymphoma. These genes and proteins provide more information about the behavior of Hodgkin lymphoma, which may help better target the lymphoma with chemotherapy or immunotherapy. Other advances. Vaccine therapy is being studied to see if it helps the body’s immune system kill cancer cells. Stem cell transplantation is being studied in combination with various chemotherapy/immunotherapy regimens for new or recurrent Hodgkin lymphoma. Mini-allogeneic or allogeneic transplantation is being tested in combination with chemotherapy/immunotherapy for new or recurrent Hodgkin lymphoma. Several new types of drugs which work in a different way than chemotherapy are also being studied. Many of these are given in tablet (pill) form. Supportive care. Clinical trials are underway to find better ways of reducing symptoms and side effects of current Hodgkin lymphoma treatments in order to improve patients’ comfort and quality of life. Learn more about common statistical terms used in cancer research. Looking for More about Current Research? If you would like additional information about the latest areas of research regarding Hodgkin lymphoma, explore these related items:
Or, choose “Next” (below, right) to continue reading this detailed section. Questions to Ask the Doctor
Talking often with the doctor is important to make informed decisions about your health care. These suggested questions are a starting point to help you learn more about your cancer care and treatment. You are also encouraged to ask additional questions that are important to you.
Patient Information Resources
In addition to Cancer.Net, there are other sources of information about this type of cancer available online. Cancer.Net maintains a list of national, not-for-profit organizations that may be helpful in finding additional information, services, and support. As always, be sure to talk with your doctor about questions you may have about information you find about this disease. View organizations that offer information on this specific type of cancer. |