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Hodgkin's Disease Overview

What Is Cancer?

Cancer is not just one disease but rather a group of diseases. All forms of cancer cause cells in the body to change and grow out of control. Most types of cancer cells form a lump or mass called a tumor. Cells from the tumor can break away and travel to other parts of the body. There they can continue to grow. This spreading process is called metastasis. When cancer spreads, it is still named after the part of the body where it started. For example, if breast cancer spreads to the lungs, it is still breast cancer, not lung cancer.

Another word for cancerous is malignant. So a cancerous tumor is called malignant. But not all tumors are cancer. A tumor that is not cancer is called benign. Benign tumors do not grow and spread the way cancer does. They are usually not a threat to life. A few cancers, such as blood cancers (leukemia), do not form a tumor.

What Is Hodgkin's Disease?

Hodgkin's disease (also called Hodgkin's lymphoma) is a type of cancer that starts in lymphatic tissue. Lymphatic tissue includes the lymph nodes and other organs that are part of the body's system that forms blood and protects against germs. Lymph nodes (sometimes called lymph glands) are small, bean-shaped organs found in many places throughout the body. The lymph nodes make and store white blood cells that fight infection. Lymph vessels, narrow tubes something like blood vessels, connect the lymph nodes. These lymph vessels carry a clear fluid that contains the white blood cells. Other parts of the lymphatic system include the spleen, the bone marrow, and the thymus. Because lymphatic tissue is found in many parts of the body, Hodgkin's disease can start almost anywhere. This disease causes the lymphatic tissue to become enlarged press on nearby structures. The cancer can spread through the lymphatic vessels. If it gets into the blood vessels it can then spread to almost any other place in the body. Hodgkin's disease is named after Dr. Thomas Hodgkin who first recognized it in 1832. There is another cancer called non-Hodgkin's lymphoma. The cancer cells of this disease look different under a microscope from the cells of Hodgkin's disease.

How Many People Get Hodgkin's Disease?

The American Cancer Society predicts that in the year 2000 there will be about 7,400 new cases of Hodgkin's disease in this country. About 1,400 people will die of the disease. Because of better treatment, death rates have fallen by more than half since the early 1970's.
Both children and adults can get Hodgkin's disease but it is most common in two age groups: early adulthood (ages 15-40), and late adulthood
(after age 55).

Treatment for most cases of Hodgkin's disease is very effective. The 1-year relative survival rate after treatment is 93%; the 5-year and 10-year rates are 82% and 72% respectively. At 15 years, the overall survival rate is 63%. The survival rate refers to the percent of people with Hodgkin's disease who live at least that many years after the cancer is diagnosed.

What Causes Hodgkin's Disease? Can It Be Prevented?

A risk factor is anything that increases a person's chance of getting disease such as cancer. Different cancers have different risk factors. Scientists have found a few risk factors that may make a person more likely to get Hodgkin's disease. There seems to be a slightly higher risk of Hodgkin's disease among people who have had "mono" (infectious mononucleosis). Some people who have reduced immune systems, for example, those with AIDS, and organ transplant patients, are also at a higher risk of Hodgkin's disease. Unlike many other types of cancer, Hodgkin's disease does not seem to be caused by something wrong with a person's genes or their diet or even something in the environment. Even if someone does have one or more risk factors for Hodgkin's disease, it's impossible to know for sure how much that risk factor contributed to causing the cancer. For the most part, we can say that no major risk factors for Hodgkin's disease have yet been found. Since we do not know what causes Hodgkin's disease is not possible to prevent the disease. A great deal of research is under way to try to find the cause of this cancer.

How Is Hodgkin's Disease Found?

Symptoms of Hodgkin's Disease There are no screening tests to find Hodgkin's disease early, and some people with the disease have no symptoms at all. When theydo occur, symptoms can include enlarged, painless lymph nodes. But in most people, especially children, enlarged lymph nodes are caused by an infection or other illness and not cancer. If you (or your child) have lymph nodes over an inch in size and no recent infection, it is best to have them checked by the doctor. Other symptoms of Hodgkin's disease can be caused by the swelling of lymph nodes inside the chest, creating pressure on the windpipe. These symptoms can include coughing or shortness of breath. Some people have fever, drenching night sweats, or weight loss. The fever can come and go over periods of several days or weeks. Itching, tiredness, and decreased appetite are other symptoms that can occur. If you or you child have these symptoms, talk to your doctor right away. And remember, there are often reasons for these symptoms other than cancer.

Tests to Diagnose Hodgkin's Disease

If there is any reason to think that a person has Hodgkin's disease, the doctor will want to conduct certain tests. The first step would be a complete medical history and thorough physical exam to see whether there is an infection. During the exam, the doctor will pay special attention to the lymph nodes. Since it is so common for people, especially children, to have swollen lymph nodes the doctor will probably prescribe antibiotics first to see if the lymph nodes shrink. If they do not, then more testing will be done. The only way to know for sure whether a person has Hodgkin's disease is to do a biopsy, that is, an examination of a tissue sample under the microscope. There are several different kinds of biopsies, and the doctor will choose the one best suited for the patient. The goal is to obtain enough tissue to make a diagnosis as quickly as possible with the fewest side effects. Some biopsies involve cutting through the skin to remove an entire node or a small part of a large tumor. In another type of biopsy, the doctor uses a thin needle to remove a small amount of fluid and tiny bits of tissue from the tumor. A doctor with special training in blood and lymphoid tissue disease examines all biopsy samples under a microscope. Sometimes the first biopsy does not provide a definite answer and more biopsies are needed.

How Is Hodgkin's Disease Staged?

Staging is the process of finding out how far the cancer has spread. This is very important because the treatment and the outlook for recovery depend on the stage of the cancer. The staging system for Hodgkin's disease is called the Ann Arbor Staging Classification. This system has four stages, labeled stage I through stage IV (1-4). In general, the lower the number, the less the cancer has spread. A higher number, such as Stage IV, means a more serious cancer. After a biopsy has confirmed that Hodgkin's disease is present, the next step is clinical staging. This consists of taking the patient's medical history, doing a physical exam, and then doing several kinds of imaging studies. Imaging studies such as CT (computed tomography) and MRI (magnetic resonance imaging) are used to create pictures of the inside of the body. They can help show the size and shape of lumps or tumors that might be cancerous. For many people, the results of the clinical staging are all that are needed to plan their treatment. Others, however, require another step called pathological staging. In this process, the doctor performs an operation called a laparotomy, checking inside the abdomen to see if any of the organs contain cancer. Small pieces of tissue are removed and looked at under a microscope to see if Hodgkin's disease is present. The spleen is also usually removed at this time.

Survival Rates by Stage of Hodgkin's Disease

The 5-year survival rate refers to the percent of patients who live at least 5 years after their cancer is treated. Many of these patients live longer than 5 years. Five-year relative survival rates are seen as more accurate because they do not include statistics for people who die of causes other than their cancer. Since these figures go back 5 years, results for people diagnosed recently can be even better since treatments are always improving.

Stage 5-year relative survival rate

I 90% to 95%
II 90% to 95%
III 85% to 90%
IV about 80%

How Is Hodgkin's DiseaseTreated?

In recent years, much progress has been made in treating Hodgkin's disease. For nearly all patients, complete cure is the goal of treatment, and about 90% of patients are cured with chemotherapy or radiation therapy. After Hodgkin's disease is staged, the doctor will discuss treatment choices with you. It is important to take time and think about all the choices. Some factors to consider include you (or your child's) overall health and the type and stage of the disease. Be sure that you understand all the risks and side effects before making a decision. The two main methods of treating Hodgkin's disease are chemotherapy, the use of cancer-killing drugs, and radiation therapy, the use of high-energy x-rays to kill cancer cells and shrink tumors. Sometimes one treatment is used and sometimes both. Bone marrow transplantation, discussed later, is used for certain people. For the most part, surgery plays little role in the treatment of Hodgkin's disease.

Chemotherapy


Chemotherapy for Hodgkin's disease can be given as a shot or a pill. Chemotherapy refers to the use of drugs to kill cancer cells. Usually the drugs are given into a vein or by mouth. Once the drugs enter the bloodstream, they spread throughout the body to destroy the cancer cells. Several drugs are given at the same time. Although the drugs kill cancer cells, they also damage normal cells, causing side effects. The exact side effects depend on the type and dose of drugs used and the length of time they are taken, but they can include hair loss, mouth sores, greater chance of infection, easy bruising or bleeding, fatigue, loss of appetite, nausea, and vomiting. These side effects are temporary and go away after treatment is finished. If you have side effects, your cancer care team can suggest steps to ease their impact. For example, other drugs can be given along with the chemotherapy to prevent or reduce nausea and vomiting. In addition, some of these drugs can have side effects that occur long after treatment is finished. These can affect a person's heart, lungs, growth, and ability to have children. There is also a risk of developing a second type of cancer. It's important to discuss these possible side effects with the doctor before treatment begins.

Radiation Therapy


Radiation therapy is the use of high-energy x-rays to destroy cancer cells. When radiation therapy is given for Hodgkin's disease, it usually involves a focused beam of radiation, given from a machine outside the body. This is known as external beam radiation. Radiation therapy is often given after 3 to 4 courses of chemotherapy. Radiation therapy can produce some serious side effects including damage to nearby healthy tissue. Other problems can include skin changes similar to sunburn, tiredness, upset stomach, and loose bowels. There can be long-term side effects as well. To reduce the risk of side effects, doctors are careful to give the exact dose needed and to aim the beam so it hits only the cancer.

Bone Marrow Transplantation

Sometimes Hodgkin's disease stops responding to standard treatment. In those cases, another type of treatment might be offered as part of a clinical trial (see below) or outside of a clinical trial. In one approach, the patient's own bone marrow is removed and stored. Then very high doses of chemotherapy (with or without radiation therapy) are given to the patient to kill the cancer. These high doses will destroy bone marrow, too. So, after the treatment, the stored marrow is given back to the patient through a vein. The bone marrow cells enter the bloodstream and return to the bone, replacing the marrow and making new red and white blood cells. Another type of transplant is called peripheral blood stem cell transplant (PBSCT). For this treatment, a machine removes the patient's blood a little at a time. Only the stem cells--immature cells from which all blood cells develop--are removed. The rest of the blood is returned to the body. This process usually takes a few hours. The stem cells arethen frozen until they are returned to the patient after treatment is finished. Doctors are finding that it is better to do these procedures earlier rather than later in the course of the disease. If the first set of treatments doesn't completely get rid of the cancer, many doctors would recommend transplantation right away. On the other hand, if a patient's disease comes back after it had gone away for a long time, most doctors would favor a second course of standard treatment before doing transplantation. What Happens After Treatment for Hodgkin's Disease
Each type of treatment for Hodgkin's disease can have side effects. Some, like loss of fertility, may be permanent. You may be able to hasten your (or your child's) recovery by being aware of the side effects before treatment starts. You might also be able to take steps to reduce the side effects and shorten the length of time that they last. Remember that every person's body is unique, and so are his or her responses and emotional needs. In some ways, each patient's cancer is like no one else's. No one can predict how each patient will respond to cancer or its treatment. Statistics can paint an overall picture, but you or your child may have special strengths such as a healthy immune system, a history of good nutrition, a strong family support system, or a deep spiritual faith. All of these have an impact on how you cope with cancer. Follow-up care is important after treatment. The health care team will explain what tests are needed and how often they should be done. Hodgkin's disease survivors will need blood tests as well as x-rays, CT scans, and other imaging studies to look for recurrent disease or long-term side effects. The good news about Hodgkin's disease is that most people with this disease are cured. The bad news is that because so many people are living for a long time, we are learning about serious side effects that happen years after the treatment is over. One of the most serious side effects of Hodgkin's disease treatment is the chance of the survivor developing a second cancer. Some kinds of chemotherapy (or chemotherapy and radiation therapy) can slightly increase the risk of the person treated developing acute leukemia later in life. But radiation therapy alone does not increase the risk for leukemia. Other types of cancer can also develop. These can occur because of radiation treatment. Young women (under age 30) who are treated with radiation to the chest have a much higher chance of developing breast cancer several years after their treatment. These women should be especially careful about following American Cancer Society guidelines for early detection of breast cancer. The guidelines stress mammography, clinical breast examination, and breast self-examination. Both men and women treated with radiation have a higher chance of developing lung cancer and thyroid cancer. Although there is no accepted screening test for lung cancer or thyroid cancer, frequent follow-up physical exams and chest x-rays may be helpful. It should be no surprise that the chance of getting lung cancer is very much higher in smokers, so not smoking is especially important among survivors of Hodgkin's disease. A less serious, but still important long-term effect of chemotherapy and radiation therapy is sterility. Men treated with nitrogen mustard lose their ability to produce sperm. Spermproduction usually returns-- but not always. Likewise, women may stop ovulating and menstruating with chemotherapy. These functions may or may not return to normal after treatment. Radiation treatment to the ovaries will permanently sterilize a woman unless the ovaries are surgically placed outside the radiation field. For unknown reasons, the immune system of people with Hodgkin's disease does not work properly. All Hodgkin's survivors should keep up with their vaccinations, including flu shots, and make sure that they get treatment for any infections. The thyroid and the heart can also be affected by treatment. Many people who have had radiation to their thyroid will develop a condition where the gland does not produce enough thyroid hormone. They will need thyroid medication. Thyroid function should be tested at least yearly. Radiation to the heart can injure the arteries that supply oxygen and nourishment to the heart muscle. In the past, people who have had radiation to the chest have had a higher rate of heart attacks. With more modern radiation treatment, this has become less a problem, but it's still important that survivors not smoke and that they follow the ACS diet guidelines to reduce their chances of heart problems.
Survivors need follow-up care for many years or decades after treatment for Hodgkin's disease. They should make a special effort to keep all appointments with the cancer care team, to follow their instructions carefully, and to report any new or recurring symptoms to the doctor right away.

Resistant and Recurrent Hodgkin's Disease

Treatment for Hodgkin's disease should get rid of all traces of the cancer. If tests show that some disease remains, the doctor might suggest trying a different kind of treatment, for example, a bone marrow transplant. If Hodgkin's disease returns at a later time, then further treatment will depend upon what kind of treatment was given initially. If chemotherapy had been used at first, different drugs might be tried. But, once radiation is given to a part of the body, it usually cannot be given again. For example, if someone had Hodgkin's disease in their chest that had been treated with radiation, more radiation could not be used if the disease came back. This holds true no matter how much time has gone by.

How Can I Learn More?

The American Cancer Society has more information about Hodgkin's disease.
Call 1-800-ACS 2345 or visit our Internet site at www.cancer.org
National Cancer Institute
Cancer Information Service 1-800-4-CANCER
Internet address: www.nci.nih.gov
The Leukemia & Lymphoma Society
1-800-955-4572 or 1-914-949-5213
Internet Address: www.leukemia-lymphoma.org

Books

A Cancer Survivor's Almanac: Charting Your Journey. Edited by Barbara Hoffman,

JD. National Coalition for Cancer Survivorship. Chronimed Publishing, 1996.

Informed Decisions: The Complete Book of Cancer Diagnosis, Treatment, and Recovery, by Gerald P.
Murphy, MD, Lois B. Morris, and Dianne Lange. The American Cancer Society. Viking, 1996.

Sexuality and Fertility After Cancer by Leslie R. Schover, PhD. John Wiley & Sons,
Inc., 1997.